Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: Clinical Profile of Four Patients and Review

Abstract

Four patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) are described. Two patients presented with sustained ventricular tachycardia and two with cardiac arrest from which they were successfully resuscitated. All four patients had typical electrocardiographic and echocardiographic features and fulfilled the task force criteria for diagnosis of ARVD/C. All four patients had T wave inversion in chest leads V1 to V4, and two had epsilon waves, and all four had premature ventricular complexes of left bundle branch block and left axis deviation pattern. All patients showed a markedly enlarged, thin and hypokinetic right ventricle. Three patients had implantable cardioverter defibrillator (ICD) implanted and are doing well. One patient who refused an ICD died suddenly 6 months after his initial presentation. Three surviving patients are on sotalol for suppression of ventricular tachycardia episodes triggering ICD shocks. A review of the existing literature on diagnosis of ARVD/C, its clinical presentation and natural history, its genetic basis, risk stratification, treatment, and prognosis is presented.