TY - JOUR
T1 - Sudden Cardiac Death Prediction in Arrhythmogenic Right Ventricular Cardiomyopathy
T2 - A Multinational Collaboration
AU - Cadrin-Tourigny, Julia
AU - Bosman, Laurens P.
AU - Wang, Weijia
AU - Tadros, Rafik
AU - Bhonsale, Aditya
AU - Bourfiss, Mimount
AU - Lie, Øyvind H.
AU - Saguner, Ardan M.
AU - Svensson, Anneli
AU - Andorin, Antoine
AU - Tichnell, Crystal
AU - Murray, Brittney
AU - Zeppenfeld, Katja
AU - Van Den Berg, Maarten P.
AU - Asselbergs, Folkert W.
AU - Wilde, Arthur A.M.
AU - Krahn, Andrew D.
AU - Talajic, Mario
AU - Rivard, Lena
AU - Chelko, Stephen
AU - Zimmerman, Stefan L.
AU - Kamel, Ihab R.
AU - Crosson, Jane E.
AU - Judge, Daniel P.
AU - Yap, Sing Chien
AU - Van Der Heijden, Jeroen F.
AU - Tandri, Harikrishna
AU - Jongbloed, Jan D.H.
AU - Van Tintelen, J. Peter
AU - Platonov, Pyotr G.
AU - Duru, Firat
AU - Haugaa, Kristina H.
AU - Khairy, Paul
AU - Hauer, Richard N.W.
AU - Calkins, Hugh
AU - Te Riele, Anneline S.J.M.
AU - James, Cynthia A.
N1 - Publisher Copyright:
© 2021 Lippincott Williams and Wilkins. All rights reserved.
PY - 2021/1/1
Y1 - 2021/1/1
N2 - 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.
AB - 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.
KW - arrhythmogenic right ventricular dysplasia
KW - calibration
KW - sudden cardiac death
KW - syncope
KW - ventricular tachycardia
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UR - http://www.scopus.com/inward/citedby.url?scp=85100280851&partnerID=8YFLogxK
U2 - 10.1161/CIRCEP.120.008509
DO - 10.1161/CIRCEP.120.008509
M3 - Article
C2 - 33296238
AN - SCOPUS:85100280851
SN - 1941-3149
VL - 14
SP - E008509
JO - Circulation: Arrhythmia and Electrophysiology
JF - Circulation: Arrhythmia and Electrophysiology
IS - 1
ER -