Sudden Cardiac Death Prediction in Arrhythmogenic Right Ventricular Cardiomyopathy: A Multinational Collaboration. (January 2021)

• Record Type: Journal Article
• Authors: Cadrin-Tourigny, Julia; Bosman, Laurens P.; Wang, Weijia; Tadros, Rafik; Bhonsale, Aditya; Bourfiss, Mimount; Lie, Øyvind H.; Saguner, Ardan M.; Svensson, Anneli; Andorin, Antoine; Tichnell, Crystal; Murray, Brittney; Zeppenfeld, Katja; van den Berg, Maarten P.; Asselbergs, Folkert W.; Wilde, Arthur A.M.; Krahn, Andrew D.; Talajic, Mario; Rivard, Lena; Chelko, Stephen; Zimmerman, Stefan L.; Kamel, Ihab R.; Crosson, Jane E.; Judge, Daniel P.; Yap, Sing-Chien; Van der Heijden, Jeroen F.; Tandri, Harikrishna; Jongbloed, Jan D.H.; van Tintelen, J. Peter; Platonov, Pyotr G.; Duru, Firat; Haugaa, Kristina H.; Khairy, Paul; Hauer, Richard N.W.; Calkins, Hugh; te Riele, Anneline S.J.M.; James, Cynthia A.;
• Is Part Of: Circulation. Volume 14:Number 1(2021)
• Publication Date: 2021-01
• Subjects: arrhythmogenic right ventricular dysplasia -- calibration -- sudden cardiac death -- syncope -- ventricular tachycardia; Arrhythmia -- Periodicals; Heart -- Electric properties -- Periodicals; 616.128
• DOI: 10.1161/CIRCEP.120.008509
• Languages: English
• ISSNs: 1941-3149

Abstract

Abstract : Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is associated with ventricular arrhythmias (VA) and sudden cardiac death (SCD). A model was recently developed to predict incident sustained VA in patients with ARVC. However, since this outcome may overestimate the risk for SCD, we aimed to specifically predict life-threatening VA (LTVA) as a closer surrogate for SCD. Methods: We assembled a retrospective cohort of definite ARVC cases from 15 centers in North America and Europe. Association of 8 prespecified clinical predictors with LTVA (SCD, aborted SCD, sustained, or implantable cardioverter-defibrillator treated ventricular tachycardia >250 beats per minute) in follow-up was assessed by Cox regression with backward selection. Candidate variables included age, sex, prior sustained VA (≥30s, hemodynamically unstable, or implantable cardioverter-defibrillator treated ventricular tachycardia; or aborted SCD), syncope, 24-hour premature ventricular complexes count, the number of anterior and inferior leads with T-wave inversion, left and right ventricular ejection fraction. The resulting model was internally validated using bootstrapping. Results: A total of 864 patients with definite ARVC (40±16 years; 53% male) were included. Over 5.75 years (interquartile range, 2.77–10.58) of follow-up, 93 (10.8%) patients experienced LTVA including 15 with SCD/aborted SCD (1.7%). Of the 8 prespecified clinical predictors, only 4 (younger age, male sex, premature ventricular complex count, and number of leads with T-wave inversion) were associated with LTVA. Notably, prior sustained VA did not predict subsequent LTVA ( P =0.850). A model including only these 4 predictors had an optimism-corrected C-index of 0.74 (95% CI, 0.69–0.80) and calibration slope of 0.95 (95% CI, 0.94–0.98) indicating minimal over-optimism. Conclusions: LTVA events in patients with ARVC can be predicted by a novel simple prediction model using only 4 clinical predictors. Prior sustained VA and the extent of functional heart disease are not associated with subsequent LTVA events. Abstract : Supplemental Digital Content is available in the text.