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. 2019 Jun 14;40(23):1850-1858.
doi: 10.1093/eurheartj/ehz103.

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

Julia Cadrin-Tourigny  1   2 , Laurens P Bosman  3   4 , Anna Nozza  5 , Weijia Wang  1 , Rafik Tadros  2 , Aditya Bhonsale  1 , Mimount Bourfiss  4 , Annik Fortier  5 , Øyvind H Lie  6   7 , Ardan M Saguner  8 , Anneli Svensson  9 , Antoine Andorin  2 , Crystal Tichnell  1 , Brittney Murray  1 , Katja Zeppenfeld  10 , Maarten P van den Berg  11 , Folkert W Asselbergs  3   4   12 , Arthur A M Wilde  13 , Andrew D Krahn  14 , Mario Talajic  2 , Lena Rivard  2 , Stephen Chelko  1 , Stefan L Zimmerman  15 , Ihab R Kamel  15 , Jane E Crosson  1 , Daniel P Judge  1 , Sing-Chien Yap  16 , Jeroen F van der Heijden  4 , Harikrishna Tandri  1 , Jan D H Jongbloed  17 , Marie-Claude Guertin  5 , J Peter van Tintelen  3   18 , Pyotr G Platonov  19 , Firat Duru  8 , Kristina H Haugaa  6   7 , Paul Khairy  2 , Richard N W Hauer  3   4 , Hugh Calkins  1 , Anneline S J M Te Riele  3   4 , Cynthia A James  1
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A new prediction model for ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy

Julia Cadrin-Tourigny et al. Eur Heart J. .

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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).

Keywords: Arrhythmogenic right ventricular cardiomyopathy; Implantable cardioverter-defibrillators; Sudden cardiac death; Ventricular arrhythmias.

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