Advanced

A new prediction model for ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy

Cadrin-Tourigny, Julia ; Bosman, Laurens P. ; Nozza, Anna ; Wang, Weijia ; Tadros, Rafik ; Bhonsale, Aditya ; Bourfiss, Mimount ; Fortier, Annik ; Lie, Øyvind H. and Saguner, Ardan M. , et al. (2019) In European Heart Journal 40(23). p.1850-1858
Abstract

AIMS: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVC) is characterized by ventricular arrhythmias (VAs) and sudden cardiac death (SCD). We aimed to develop a model for individualized prediction of incident VA/SCD in ARVC patients. METHODS AND RESULTS: Five hundred and twenty-eight patients with a definite diagnosis and no history of sustained VAs/SCD at baseline, aged 38.2 ± 15.5 years, 44.7% male, were enrolled from five registries in North America and Europe. Over 4.83 (interquartile range 2.44-9.33) years of follow-up, 146 (27.7%) experienced sustained VA, defined as SCD, aborted SCD, sustained ventricular tachycardia, or appropriate implantable cardioverter-defibrillator (ICD) therapy. A prediction model estimating... (More)

AIMS: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVC) is characterized by ventricular arrhythmias (VAs) and sudden cardiac death (SCD). We aimed to develop a model for individualized prediction of incident VA/SCD in ARVC patients. METHODS AND RESULTS: Five hundred and twenty-eight patients with a definite diagnosis and no history of sustained VAs/SCD at baseline, aged 38.2 ± 15.5 years, 44.7% male, were enrolled from five registries in North America and Europe. Over 4.83 (interquartile range 2.44-9.33) years of follow-up, 146 (27.7%) experienced sustained VA, defined as SCD, aborted SCD, sustained ventricular tachycardia, or appropriate implantable cardioverter-defibrillator (ICD) therapy. A prediction model estimating annual VA risk was developed using Cox regression with internal validation. Eight potential predictors were pre-specified: age, sex, cardiac syncope in the prior 6 months, non-sustained ventricular tachycardia, number of premature ventricular complexes in 24 h, number of leads with T-wave inversion, and right and left ventricular ejection fractions (LVEFs). All except LVEF were retained in the final model. The model accurately distinguished patients with and without events, with an optimism-corrected C-index of 0.77 [95% confidence interval (CI) 0.73-0.81] and minimal over-optimism [calibration slope of 0.93 (95% CI 0.92-0.95)]. By decision curve analysis, the clinical benefit of the model was superior to a current consensus-based ICD placement algorithm with a 20.6% reduction of ICD placements with the same proportion of protected patients (P < 0.001). CONCLUSION: Using the largest cohort of patients with ARVC and no prior VA, a prediction model using readily available clinical parameters was devised to estimate VA risk and guide decisions regarding primary prevention ICDs (www.arvcrisk.com).

(Less)
Please use this url to cite or link to this publication:
author
, et al. (More)
(Less)
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Arrhythmogenic right ventricular cardiomyopathy, Implantable cardioverter-defibrillators, Sudden cardiac death, Ventricular arrhythmias
in
European Heart Journal
volume
40
issue
23
pages
9 pages
publisher
Oxford University Press
external identifiers
  • pmid:30915475
  • scopus:85068197100
ISSN
1522-9645
DOI
10.1093/eurheartj/ehz103
language
English
LU publication?
yes
id
64991361-5d8a-43ec-90a3-a9798805f4d7
date added to LUP
2019-07-09 14:54:47
date last changed
2020-07-08 04:59:47
@article{64991361-5d8a-43ec-90a3-a9798805f4d7,
  abstract     = {<p>AIMS: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVC) is characterized by ventricular arrhythmias (VAs) and sudden cardiac death (SCD). We aimed to develop a model for individualized prediction of incident VA/SCD in ARVC patients. METHODS AND RESULTS: Five hundred and twenty-eight patients with a definite diagnosis and no history of sustained VAs/SCD at baseline, aged 38.2 ± 15.5 years, 44.7% male, were enrolled from five registries in North America and Europe. Over 4.83 (interquartile range 2.44-9.33) years of follow-up, 146 (27.7%) experienced sustained VA, defined as SCD, aborted SCD, sustained ventricular tachycardia, or appropriate implantable cardioverter-defibrillator (ICD) therapy. A prediction model estimating annual VA risk was developed using Cox regression with internal validation. Eight potential predictors were pre-specified: age, sex, cardiac syncope in the prior 6 months, non-sustained ventricular tachycardia, number of premature ventricular complexes in 24 h, number of leads with T-wave inversion, and right and left ventricular ejection fractions (LVEFs). All except LVEF were retained in the final model. The model accurately distinguished patients with and without events, with an optimism-corrected C-index of 0.77 [95% confidence interval (CI) 0.73-0.81] and minimal over-optimism [calibration slope of 0.93 (95% CI 0.92-0.95)]. By decision curve analysis, the clinical benefit of the model was superior to a current consensus-based ICD placement algorithm with a 20.6% reduction of ICD placements with the same proportion of protected patients (P &lt; 0.001). CONCLUSION: Using the largest cohort of patients with ARVC and no prior VA, a prediction model using readily available clinical parameters was devised to estimate VA risk and guide decisions regarding primary prevention ICDs (www.arvcrisk.com).</p>},
  author       = {Cadrin-Tourigny, Julia and Bosman, Laurens P. and Nozza, Anna and Wang, Weijia and Tadros, Rafik and Bhonsale, Aditya and Bourfiss, Mimount and Fortier, Annik and Lie, Øyvind 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 Guertin, Marie Claude 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         = {1522-9645},
  language     = {eng},
  number       = {23},
  pages        = {1850--1858},
  publisher    = {Oxford University Press},
  series       = {European Heart Journal},
  title        = {A new prediction model for ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy},
  url          = {http://dx.doi.org/10.1093/eurheartj/ehz103},
  doi          = {10.1093/eurheartj/ehz103},
  volume       = {40},
  year         = {2019},
}