Gabriela M Orgeron1, Anneline Te Riele1, Crystal Tichnell1, Weijia Wang1, Brittney Murray1, Aditya Bhonsale1, Daniel P Judge1, Ihab R Kamel1, Stephan L Zimmerman1, Harikrishna Tandri1, Hugh Calkins1, Cynthia A James2. 1. From the Department of Medicine, Division of Cardiology (G.M.O., C.T., W.W., B.M., A.B., D.P.J., H.T., H.C., C.A.J.) and Department of Radiology (I.R.K., S.L.Z.), Johns Hopkins Hospital, Baltimore, MD; and Department of Heart and Lungs, Division of Cardiology, University Medical Center Utrecht, and Netherlands Heart Institute (A.t.R.). 2. From the Department of Medicine, Division of Cardiology (G.M.O., C.T., W.W., B.M., A.B., D.P.J., H.T., H.C., C.A.J.) and Department of Radiology (I.R.K., S.L.Z.), Johns Hopkins Hospital, Baltimore, MD; and Department of Heart and Lungs, Division of Cardiology, University Medical Center Utrecht, and Netherlands Heart Institute (A.t.R.). cjames7@jhmi.edu.
Abstract
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 <240 ms was also assessed. Two hundred twenty-four (61%) patients had a Class I implantable cardioverter-defibrillator indication; 80 (22%), Class IIa; 54 (15%), Class IIb; and 7 (2%), Class III. During a median 4.2 (interquartile range, 1.7-8.4)-year follow-up, 190 (52%) patients had VT/VF and 60 (16%) had ventricular fibrillation/flutter. Although the algorithm appropriately differentiated risk of VT/VF, incidence of VT/VF was underestimated (observed versus expected: 29.6 [95% confidence interval, 25.2-34.0] versus >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 (<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.
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/cardiomyopathypatients 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 <240 ms was also assessed. Two hundred twenty-four (61%) patients had a Class I implantable cardioverter-defibrillator indication; 80 (22%), Class IIa; 54 (15%), Class IIb; and 7 (2%), Class III. During a median 4.2 (interquartile range, 1.7-8.4)-year follow-up, 190 (52%) patients had VT/VF and 60 (16%) had ventricular fibrillation/flutter. Although the algorithm appropriately differentiated risk of VT/VF, incidence of VT/VF was underestimated (observed versus expected: 29.6 [95% confidence interval, 25.2-34.0] versus >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 (<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.
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