The clinical course and long-term follow-up of 12 patients with arrhythmogenic right ventricular dysplasia causing ventricular tachycardia are presented. No patient had a history of congestive heart failure and the cardiothoracic ratio measured less than or equal to 0.5 in all patients. All 12 patients were symptomatic during ventricular tachycardia; syncope occurred in 4. Exercise-related symptoms were present in 8 (73%) of 11 patients. The mean right ventricular ejection fraction was 31% (range 20% to 54%), and the mean left ventricular ejection fraction was 68% (range 44% to 88%). Signal averaging of the rest electrocardiogram (ECG) revealed late potentials in five of eight patients. During programmed electrical stimulation, sustained or nonsustained ventricular tachycardia showing a left bundle branch block configuration was induced in all patients. One patient underwent right ventricular disconnection and died 1 week after operation of low cardiac output failure. The remaining 11 patients were all treated medically and are alive at a mean follow-up time of 7.9 years after the onset of symptoms. Recurrence of symptomatic and documented sustained monomorphic ventricular tachycardia occurred in eight patients and could not be predicted by results of long-term ECG monitoring, treadmill exercise testing or programmed stimulation. In conclusion, despite recurrence of ventricular tachycardia, patients with arrhythmogenic right ventricular dysplasia have a favorable outcome when treated medically. Noninvasive studies (imaging techniques, ambulatory ECG monitoring and exercise testing) provide data that may be sufficient in diagnosing arrhythmogenic right ventricular dysplasia.