Arrhythmogenic right ventricular dysplasia: electrophysiologic and morphologic features in nine patients

Arrhythmogenic right ventricular dysplasia is a potentially life-threatening disorder, not previously well described in India. We analysed the electrocardiographic, electrophysiologic, angiographic, signal-averaged electrocardiogram and magnetic resonance imaging features of nine patients having arrhythmogenic right ventricular dysplasia at our centre. There were seven males and two females, aged 25 to 55 years. Eight patients presented with sustained monomorphic ventricular tachycardia. The electrocardiogram showed prolonged QRS duration in four, T inversion in leads V2-V3 in four, and epsilon wave in two patients. Abnormal late potentials were present in eight patients. During electrophysiologic study in seven patients, 13 different VTs were induced. Seven patients had right ventricular dilatation and dysfunction and left ventricular involvement was seen in three patients. Eight patients were treated with amiodarone, including one who received an implantable cardioverter-defibrillator. At a follow-up of 8.6 +/- 5.3 months, there was no death, while one patient had recurrence of ventricular tachycardia. Arrhythmogenic right ventricular dysplasia seems to predominantly affect middle-aged men, the presentation most often being sustained monomorphic ventricular tachycardia. Depolarisation and/or repolarisation abnormalities in the electrocardiogram are commonly present. Multiple ventricular tachycardia morphologies during electrophysiologic study and abnormal right ventricular angiograms are usually observed.