MYOCARDIAL DISEASE (A ABBATE, SECTION EDITOR) Radiofrequency Ablation in Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Jorge Romero 1 & Michael Grushko 1 & David F. Briceño 1 & Andrea Natale 2 & Luigi Di Biase 1 Published online: 5 August 2017 # Springer Science+Business Media, LLC 2017 Abstract Purpose of Review Arrhythmogenic right ventricular cardio- myopathy (ARVC) is a heritable form of cardiomyopathy, typically with autosomal dominant transmission, shown to be a defect in the cardiac desmosomes, with distinct regional and histopathological features. Clinically, this can ultimately result in bi-ventricular failure and/or malignant ventricular tachycardia (VT) via reentrant circuits created by patchy scar formation. We sought to review the current treatment for ven- tricular arrhythmias in the setting of ARVC, with particular attention to radiofrequency (RF) ablation and its varied tech- niques, along with potential therapies in the ablation spectrum. Recent Findings There is underwhelming data on the effec- tiveness of medical therapy for ARVC-related VT, including beta-blockers and antiarrhythmic medication. Primary and secondary prophylactic implantable-cardioverter defibrillator (ICD) implantation in higher-risk patients is recommended. More recently, RF ablation has been used for ARVC-related VT. Endocardial VT ablation in this setting can produce acute success, though recurrence rate is quite high, which may be explained by the more epicardial and patchy nature of the disease. Combined endocardial-epicardial ablation has since been shown to be feasible, safe, and with significantly better acute and long-term success, particularly when combined with scar dechanneling or homogenization of the scar. However, recurrence rates are not insignificant, and ablation does not eliminate the need for ICD placement. Summary Medical therapy for ARVC-related VT is subopti- mal. RF ablation techniques including endocardial and epicar- dial approaches appear to have the highest success rates for ARVC-related VT. Catheter ablation of VT in ARVC patients should be considered a potentially effective strategy for elim- inating frequent VT episodes and ICD shocks rather than a curative therapeutic approach, until long-term efficacy has been consistently documented. Research into the optimal mapping and ablation techniques are promising and ongoing. Keywords ARVD . Ventricular tachycardia . Catheter ablation . Cardiomyopathy . ICD . Epicardial access Introduction Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable form of cardiomyopathy, genetically determined predominantly via an autosomal dominant pattern. The prev- alence of ARVC is approximately 1:5000, affecting men more frequently than women with a ratio of 3:1. ARVC predomi- nantly affects the right ventricle (RV), but may also involve the left ventricle (LV) and results in heart failure and ventric- ular tachycardia (VT) [1]. In 1982, Marcus et al. initially de- scribed the disease, when he reported 24 cases of VT with left bundle branch block (LBBB) morphology, RV wall motion abnormalities, and replacement of the RV myocardium by adipose and fibrous tissue [2]. Later, in 1984, the electrocar- diographic characteristics of ARVC were described, including the pathognomonic epsilon wave [3]. Pathologically, ARVC is characterized by fibro-fatty replacement, myocardial atrophy, fibrosis, and eventually thinning of the RV wall with RV di- latation and formation of small aneurysms in this chamber [4]. This article is part of the Topical Collection on Myocardial Disease * Luigi Di Biase dibbia@gmail.com 1 Division of Cardiology at Montefiore-Einstein Center for Heart and Vascular Care, Montefiore Medical Center, Albert Einstein College of Medicine, 111 East 210th Street, Bronx, NY 10467, USA 2 Texas Cardiac Arrhythmia Institute, Austin, Texas, USA Curr Cardiol Rep (2017) 19: 82 DOI 10.1007/s11886-017-0893-3