[Prognosis and long-term development of arrhythmogenic dysplasia of the right ventricle]

The authors studied the clinical, angiographic and electrophysiological features, and the long-term outcome of patients with arrhythmogenic right ventricular dysplasia (ARVD). The study was retrospective and involved 22 patients (3 women and 19 men) with an average age of 36 years at the time of their first documented ventricular tachycardia (VT) and admitted to hospital between 1971 and 1989. The diagnosis of ARVD was based on the association of monomorphic VT with a left bundle branch block morphology and right ventricular abnormalities on angiography. Fifteen patients were treated by antiarrhythmic drugs alone, 5 by antiarrhythmic drugs and tachycardia ablation and 2 by antiarrhythmic drugs and cardiac transplantation. An automatic defibrillator was implanted in one patient. After the first episode of VT, the beginning of follow-up, the criteria of evaluation were mortality, right heart failure and the evolution of the arrhythmia. The study population had the following characteristics. Familial forms or ARVD were observed in 18%. The mitral ventricular arrhythmia was often induced by exercises (45%). Twenty-one patients had a sustained VT and 1 patient had non-sustained VT. The resting ECG in sinus rhythm was abnormal in all cases. Thirty-two different VT morphologies were recorded. The abnormalities of the right ventricular wall motion on ventriculography were localised in 45% of cases and diffuse in 55% of cases. Seventy per cent of patients undergoing electrophysiological investigation had inducible sustained VT. The clinical outcome after an average of 10.7 years' follow-up was: 17 survivors, 3 sudden deaths and 4 cases of right ventricular failure. The 10 year actuarial survival rate was 75%.(ABSTRACT TRUNCATED AT 250 WORDS)