Arrhythmogenic right ventricular dysplasia

Arrhythmogenic right ventricular dysplasia is a cardiomyopathy with autopsy evidence of fibrous and fatty infiltration of the right ventricular. The disease, which shows familial clustering, causes electrical instability that may place affected subjects at risk of sudden death. It is characterized by ECG changes and right ventricular dysfunction. Death can occur due to ventricular arrhythmia. We describe a 46-year-old female Cypriot who presented with recurrent syncope and palpitation due to ventricular tachycardia with the LBBB pattern; T waves were inverted in V1-V3. Echocardiogram and cardiac catheterization revealed severe right ventricular dysfunction. Her sister had died several years before of a similar syndrome, including ventricular tachycardia. Family members should be screened for right ventricular dysplasia. Those currently asymptomatic should be given prophylactic therapy to prevent sudden death. Treatment is either medical: sotalol, beta-blockers or verapamil; or surgical: ablation of the arrhythmogenic focus in the right ventricle, total disconnection of the right ventricular free wall; or implantation of a cardioverter defibrillator.