Consensus on Arrhythmogenic RV Cardiomyopathy Treatment

Study Questions:

What are the current recommendations for risk stratification and therapy for arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D)?


An international task force summarizes the current evidence and provides recommendations in this comprehensive overview of risk stratification and therapy for ARVC/D.


Recommendations are presented for risk stratification, lifestyle changes, pharmacologic therapy, heart failure management, catheter ablation, and implantable-cardioverter defibrillator (ICD) therapy. The key points from these recommendations include: 1) consideration of electrophysiology study in the evaluation of ARVC/D (Class IIa); 2) avoidance of competitive or endurance sports (Class I); 3) use of antiarrhythmic drugs to decrease appropriate ICD therapy (Class I); 4) avoidance of antiarrhythmic drugs in healthy gene carriers (Class III); 5) use of beta-blockers for patients with arrhythmias (Class I) and potentially in all patients with ARVC/D (Class IIa); 6) standard pharmacologic treatment for associated heart failure (Class I); 7) catheter ablation, including epicardial ablation, as an adjunct to ICD therapy (Class I), but not as an alternative to ICD for prevention of sudden cardiac death (SCD); 8) ICD therapy in patients with ≥1 episode of hemodynamically unstable sustained ventricular tachycardia (VT)/ventricular fibrillation (VF) or severe systolic dysfunction (Class I); 9) consider ICD therapy in patients with major risk factors (syncope, nonsustained VT, moderate ventricular dysfunction); and 10) avoidance of ICD therapy in asymptomatic patients without risk factors.


Risk stratification and management of ARVC/D has progressed, but much more data are needed. Current strategies are palliative.


The task force provides a useful summary of existing evidence and a straightforward set of recommendations aimed to provide adequate prevention of SCD in those at risk, yet avoid overtreatment in asymptomatic or healthy gene carriers.

Clinical Topics: Arrhythmias and Clinical EP, Heart Failure and Cardiomyopathies, Implantable Devices, EP Basic Science, Genetic Arrhythmic Conditions, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Acute Heart Failure

Keywords: Anti-Arrhythmia Agents, Arrhythmias, Cardiac, Arrhythmogenic Right Ventricular Dysplasia, Cardiomyopathies, Catheter Ablation, Death, Sudden, Cardiac, Defibrillators, Implantable, Electrophysiology, Heart Failure, Risk Factors, Tachycardia, Ventricular, Ventricular Dysfunction, Ventricular Fibrillation

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