Arrhythmogenic right ventricular dysplasia. Clinical, electrophysiological, and pathological features

Arrhythmogenic right ventricular dysplasia (ARVD) describes the syndrome of recurrent ventricular tachycardia of right ventricular origin and cardiomyopathic changes of the right ventricle. We report the clinical, electrophysiologic, and angiographic characteristics of four patients who presented with ventricular tachycardia of left bundle branch block configuration, and in whom the right ventricular origin of tachycardia was confirmed by endocardial mapping, and a diagnosis of ARVD was substantiated by histological examination. ARVD should be suspected in all patients with ventricular tachycardia of left bundle branch block configuration, especially in young adults with an otherwise normal heart. Once suspected, diagnosis can often be established by non-invasive investigation. Surgical treatment may be difficult because of the diffuse nature of right ventricular involvement.