Sudden Cardiac Death Prediction in Arrhythmogenic Right Ventricular Cardiomyopathy A Multinational Collaboration

Julia Cadrin-Tourigny*, Laurens P. Bosman, Weijia Wang, Rafik Tadros, Aditya Bhonsale, Mimount Bourfiss, Oyvind H. Lie, Ardan M. Saguner, Anneli Svensson, Antoine Andorin, Crystal Tichnell, Brittney Murray, Katja Zeppenfeld, Maarten P. van den Berg, Folkert W. Asselbergs, Arthur A. M. Wilde, Andrew D. Krahn, Mario Talajic, Lena Rivard, Stephen ChelkoStefan L. Zimmerman, Ihab R. Kamel, Jane E. Crosson, Daniel P. Judge, Sing-Chien Yap, Jeroen F. Van der Heijden, Harikrishna Tandri, Jan D. H. Jongbloed, J. Peter van Tintelen, Pyotr G. Platonov, Firat Duru, Kristina H. Haugaa, Paul Khairy, Richard N. W. Hauer, Hugh Calkins, Anneline S. J. M. te Riele, Cynthia A. James

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

79 Citations (Scopus)
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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.


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.


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.


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.

Original languageEnglish
Number of pages11
JournalCirculation. Arrhythmia and Electrophysiology
Issue number1
Early online date9-Dec-2020
Publication statusPublished - Jan-2021


  • arrhythmogenic right ventricular dysplasia
  • calibration
  • sudden cardiac death
  • syncope
  • ventricular tachycardia


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