Arrhythmogenic Right Ventricular Cardiomyopathy and Therapeutical Approaches

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by fibro-lipomatous infiltration of right ventricular free wall and ventricular tachycardia with left bundle branch block morphology in young adults. Clinical manifestations include structural and functional abnormalities of right ventricle and arrhythmias leading to sudden death. The most common cause of death is ventricular tachycardia. Retrospective evaluation of young sudden deaths and screening of their families have revealed that ARVC has wide clinical and pathologic spectrum and more important place in the etiology of sudden death. Recently as a result of advances in genetic technology, chromosomal abnormalities responsible for disease were identified. Electrocardiographic, echocardiographic, magnetic resonance imaging and right heart catheterization features and diagnostic criteria of the disease are well defined. Although a lot of therapeutical implications have been used to improve survival and to provide a better quality of life, it seems impossible to have cure with today's therapeutical modalities. According to the severity and the extent of right ventricular disease, pharmacological and non-pharmacological therapies including surgery, radiofrequency ablation and implantable cardioverter defibrillators (ICD) have been used to prevent ventricular tachycardia and sudden death in patients with ARVC. Since ARVC is a progressive disease and has a high recurrence rate with medical, surgical and ablation therapies, ICD implantation will play more important role in preventing of ventricular tachycardia and sudden death in the future. The purpose of this article is to review the clinical manifestations, recently defined genetic aspects, diagnosis, prognosis and new treatment modalites of ARVC.