RIGHT-VENTRICULAR CARDIOMYOPATHY IN IDENTICAL AND NONIDENTICAL YOUNG TWINS

We describe the first sets of identical and nonidentical twins with right ventricular cardiomyopathy (RVC). Pair A: A 12-year-old boy was referred because of palpitation and syncope. Clinical and instrument examinations revealed an enlarged and depressed right ventricle (end-diastolic volume = 110 Ml/M2; ejection fraction = 44%), spontaneous ventricular tachycardia, and fatty-fibrous infiltrates in the biopsy specimens. His asymptomatic, monozygotic twin showed localized involvement of the right ventricle with isolated, ventricular extrasystoles. Pair B. These 18-year-old nonidentical twin boys showed diffuse right ventricular involvement (end-diastolic volume = 110 Ml/M2 and 114 Ml/M2; ejection fraction = 30% and 24%, respectively), induction of sustained and nonsustained ventricular tachycardia, respectively, and fibrosis on endomyocardial biopsy. One of the boys died suddenly at rest after documented ventricular fibrillation. These cases support the hypothesis of a genetic etiology with a minor role for genotype and point to the important influence of environmental factors in determining the clinical features of the disease.