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

Julia Cadrin-Tourigny, Laurens P. Bosman, Anna Nozza, Weijia Wang, Rafik Tadros, Aditya Bhonsale, Mimount Bourfiss, Annik Fortier, Øyvind 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 TalajicLena Rivard, Stephen Chelko, Stefan 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, Marie Claude Guertin, 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

Research output: Contribution to journalArticle

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 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).

Original languageEnglish (US)
Pages (from-to)1850-1858
Number of pages9
JournalEuropean heart journal
Volume40
Issue number23
DOIs
StatePublished - Jun 14 2019

Fingerprint

Arrhythmogenic Right Ventricular Dysplasia
Cardiac Arrhythmias
Sudden Cardiac Death
Implantable Defibrillators
Ventricular Tachycardia
Stroke Volume
Confidence Intervals
Ventricular Premature Complexes
Decision Support Techniques
Syncope
Primary Prevention
North America
Calibration
Registries
Consensus

Keywords

  • Arrhythmogenic right ventricular cardiomyopathy
  • Implantable cardioverter-defibrillators
  • Sudden cardiac death
  • Ventricular arrhythmias

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

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.; 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.; Guertin, Marie Claude; 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.

In: European heart journal, Vol. 40, No. 23, 14.06.2019, p. 1850-1858.

Research output: Contribution to journalArticle

Cadrin-Tourigny, J, Bosman, LP, Nozza, A, Wang, W, Tadros, R, Bhonsale, A, Bourfiss, M, Fortier, A, Lie, ØH, Saguner, AM, Svensson, A, Andorin, A, Tichnell, C, Murray, B, Zeppenfeld, K, van den Berg, MP, Asselbergs, FW, Wilde, AAM, Krahn, AD, Talajic, M, Rivard, L, Chelko, S, Zimmerman, SL, Kamel, IR, Crosson, JE, Judge, DP, Yap, SC, van der Heijden, JF, Tandri, H, Jongbloed, JDH, Guertin, MC, van Tintelen, JP, Platonov, PG, Duru, F, Haugaa, KH, Khairy, P, Hauer, RNW, Calkins, H, Te Riele, ASJM & James, CA 2019, 'A new prediction model for ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy', European heart journal, vol. 40, no. 23, pp. 1850-1858. https://doi.org/10.1093/eurheartj/ehz103
Cadrin-Tourigny J, Bosman LP, Nozza A, Wang W, Tadros R, Bhonsale A et al. A new prediction model for ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy. European heart journal. 2019 Jun 14;40(23):1850-1858. https://doi.org/10.1093/eurheartj/ehz103
Cadrin-Tourigny, Julia ; Bosman, Laurens P. ; Nozza, Anna ; Wang, Weijia ; Tadros, Rafik ; Bhonsale, Aditya ; Bourfiss, Mimount ; Fortier, Annik ; 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. ; Guertin, Marie Claude ; 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. / A new prediction model for ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy. In: European heart journal. 2019 ; Vol. 40, No. 23. pp. 1850-1858.
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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 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).",
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TY - JOUR

T1 - A new prediction model for ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy

AU - Cadrin-Tourigny, Julia

AU - Bosman, Laurens P.

AU - Nozza, Anna

AU - Wang, Weijia

AU - Tadros, Rafik

AU - Bhonsale, Aditya

AU - Bourfiss, Mimount

AU - Fortier, Annik

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 - Guertin, Marie Claude

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.

PY - 2019/6/14

Y1 - 2019/6/14

N2 - 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).

AB - 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).

KW - Arrhythmogenic right ventricular cardiomyopathy

KW - Implantable cardioverter-defibrillators

KW - Sudden cardiac death

KW - Ventricular arrhythmias

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