Treatment of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: An International Task Force Consensus Statement

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inheritable heart muscle disease that predominantly affects the right ventricle (RV) and predisposes to ventricular arrhythmias and sudden cardiac death (SCD).1–17 In the last three decades, there have been a significant number of studies defining the pathogenesis, genetic aspects, and clinical manifestations of the disease (See ‘Etiology, pathogenesis, diagnosis and natural history’ in the online-only Data Supplement). In 1994 and 2010, an International Task Force (ITF) document proposed guidelines for the standardized diagnosis of ARVC/D based on electrocardiographic (ECG), arrhythmic, morphological, histopathologic, and clinico-genetic factors.18,19 The growing knowledge regarding arrhythmic outcome, risk factors, and life-saving therapeutic interventions, make it particularly timely to critically address and place into perspective the issues relevant to the clinical management of ARVC/D patients. The present ITF consensus statement is a comprehensive overview of currently used risk stratification algorithms and approaches to therapy, either pharmacological or nonpharmacological, which often poses a clinical challenge to cardiovascular specialists and other practitioners, particularly those infrequently engaged in the management of ARVC/D. This document should be regarded as a guide to clinical practice where rigorous evidence is still lacking, because of the relatively low disease prevalence and the absence of controlled studies. Recommendations are based on available data derived from nonrandomized and observational studies and consensus within the conference panellists. When development of prognostic-therapeutic algorithms was controversial, management decisions were recommended to be individualized. Recommendation and level of evidence of specific management options were classified according to predefined scales, as outlined in Tables 1 and 2 (http://www.escardio.org/guidelines-surveys/esc-guidelines/about/Pages/rules-writing.aspx). Because randomized studies are not available, most consensus recommendations on treatment of ARVC/D are based on data derived from follow-up registries and/or experts opinions (ie, level of evidence B or C). View this table: Table 1. Classes of Recommendations View this table: Table 2. Levels of Evidence All members of …