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Background: Ventricular arrhythmias are a feared complication of arrhythmogenic right ventricular dysplasia/cardiomyopathy. In 2015, an International Task Force Consensus Statement proposed a risk stratification algorithm for implantable cardioverter-defibrillator placement in arrhythmogenic right ventricular dysplasia/cardiomyopathy. Methods and Results: To evaluate performance of the algorithm, 365 arrhythmogenic right ventricular dysplasia/cardiomyopathy patients were classified as having a Class I, IIa, IIb, or III indication per the algorithm at baseline. Survival free from sustained ventricular arrhythmia (VT/VF) in follow-up was the primary outcome. Incidence of ventricular fibrillation/flutter cycle length  10%/year Class I; 15.5 [confidence interval 11.1–21.6] versus 1% to 10%/year Class IIa). In addition, the algorithm did not differentiate survival free from ventricular fibrillation/flutter between Class I and IIa patients (P =0.97) or for VT/VF in Class I and IIa primary prevention patients (P =0.22). Adding Holter results (&lt;1000 premature ventricular contractions/24 hours) to International Task Force Consensus classification differentiated risks.Conclusions: While the algorithm differentiates arrhythmic risk well overall, it did not distinguish ventricular fibrillation/flutter risks of patients with Class I and IIa implantable cardioverter-defibrillator indications. Limited differentiation was seen for primary prevention cases. As these are vital uncertainties in clinical decision-making, refinements to the algorithm are suggested prior to implementation.

circulation. arrhythmia and electrophysiology

Performance of the 2015 International Task Force Consensus Statement Risk Stratification Algorithm for Implantable Cardioverter-Defibrillator Placement in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy