Arrhythmogenic Right Ventricular Dysplasia

Abstract

Arrhythmogenic right ventricular dysplasia is a cardiomyopathy of young that presents with a sudden cardiac death. It had unique features that stimulated different classification, based on combination of right ventricular imaging by echocardiography and cardiac magnetic resonance imaging, histology, repolarization abnormalities, depolarization and conduction abnormalities, arrhythmias and family history (including genetic testing). Hence the usual Investigation pathway requires multimodality approach. Treatment is complex that starts with exercise restriction in these active young population and end up with insertion of Automated Implantable Cardioverter Defibrillator with its own morbidity. Antiarrhythmic drugs are eliminating frequent Ventricular Tachycardia episodes and Defibrillator shocks rather than a curative therapy. There had been a great progress in studying the underline genetic part but the field still wide open. This review will focus on the recent update in Arrhythmogenic right ventricular dysplasia