Follow-up was obtained in 33 of 35 patients with arrhythmogenic right ventricular disease (ARVD) from three centers, one in France, and two in the United States. The majority of patients are living but require antiarrhythmic drug therapy. Analysis of syncope as a prognostic factor for arrhythmic death in this series, as well as in patients with ARVD reported in the literature, suggests a more favourable long-term prognosis in those patients who did not have a history of syncope. ARVD may be due to a variety of pathological entities and the prognosis of patients who have different aetiologies may well be distinct from one another.