Skip to main content

Lund University Publications

LUND UNIVERSITY LIBRARIES

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

Cadrin-Tourigny, Julia ; Bosman, Laurens P. ; Wang, Weijia ; Tadros, Rafik ; Bhonsale, Aditya ; Bourfiss, Mimount ; Lie, Oyvind H. ; Saguner, Ardan M. ; Svensson, Anneli and Andorin, Antoine , et al. (2021) In Circulation: Arrhythmia and Electrophysiology 14(1).
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... (More)

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.

(Less)
Please use this url to cite or link to this publication:
author
; ; ; ; ; ; ; ; and , et al. (More)
; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; and (Less)
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
arrhythmogenic right ventricular dysplasia, calibration, sudden cardiac death, syncope, ventricular tachycardia
in
Circulation: Arrhythmia and Electrophysiology
volume
14
issue
1
pages
11 pages
publisher
Lippincott Williams & Wilkins
external identifiers
  • scopus:85100280851
  • pmid:33296238
ISSN
1941-3149
DOI
10.1161/CIRCEP.120.008509
language
English
LU publication?
yes
id
21046c62-800d-4861-9888-db91f59651c8
date added to LUP
2022-03-03 17:36:32
date last changed
2024-06-13 11:00:06
@article{21046c62-800d-4861-9888-db91f59651c8,
  abstract     = {{<p>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 &gt;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.</p>}},
  author       = {{Cadrin-Tourigny, Julia and Bosman, Laurens P. and Wang, Weijia and Tadros, Rafik and Bhonsale, Aditya and Bourfiss, Mimount and Lie, Oyvind H. and Saguner, Ardan M. and Svensson, Anneli and Andorin, Antoine and Tichnell, Crystal and Murray, Brittney and Zeppenfeld, Katja and Van Den Berg, Maarten P. and Asselbergs, Folkert W. and Wilde, Arthur A.M. and Krahn, Andrew D. and Talajic, Mario and Rivard, Lena and Chelko, Stephen and Zimmerman, Stefan L. and Kamel, Ihab R. and Crosson, Jane E. and Judge, Daniel P. and Yap, Sing Chien and Van Der Heijden, Jeroen F. and Tandri, Harikrishna and Jongbloed, Jan D.H. and Van Tintelen, J. Peter and Platonov, Pyotr G. and Duru, Firat and Haugaa, Kristina H. and Khairy, Paul and Hauer, Richard N.W. and Calkins, Hugh and Te Riele, Anneline S.J.M. and James, Cynthia A.}},
  issn         = {{1941-3149}},
  keywords     = {{arrhythmogenic right ventricular dysplasia; calibration; sudden cardiac death; syncope; ventricular tachycardia}},
  language     = {{eng}},
  number       = {{1}},
  publisher    = {{Lippincott Williams & Wilkins}},
  series       = {{Circulation: Arrhythmia and Electrophysiology}},
  title        = {{Sudden Cardiac Death Prediction in Arrhythmogenic Right Ventricular Cardiomyopathy : A Multinational Collaboration}},
  url          = {{http://dx.doi.org/10.1161/CIRCEP.120.008509}},
  doi          = {{10.1161/CIRCEP.120.008509}},
  volume       = {{14}},
  year         = {{2021}},
}