Role of Mapping in Arrhythmogenic Right Ventricular Cardiomyopathy

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD) is a genetic cardiomyopathy characterized by progressive replacement of right ventricular myocardium with fatty and fibrous tissue [1, 2]. The prevalence of ARVD has been estimated to be 1 in 5000 in the United States. Ventricular tachycardia (VT) is the most feared complication in patients with ARVD. The main objective of ARVD management is to prevent sudden cardiac death and symptomatic ventricular arrhythmias. Implantable cardioverter defibrillators remain the cornerstone of strategies to prevent sudden cardiac death in this patient population. Antiarrhythmic drugs and catheter ablation are used primarily as adjunctive procedures in patients with ARVD who are experiencing frequent symptomatic episodes of nonsustained or sustained ventricular arrhythmias The purpose of this chapter is twofold. Firstly, we seek to provide a concise review of the approaches to diagnosis and management of patients with ARVD. Secondly, we seek to review the role of electrical and electroanatomic mapping in both diagnosis and management of patients with ARVD.