Results:

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.