Programmed Ventricular Stimulation as an Additional Primary Prevention Risk Stratification Tool in Arrhythmogenic Right Ventricular Cardiomyopathy: A Multinational Study

Alessio Gasperetti, Richard T. Carrick, Sarah Costa, Paolo Compagnucci, Laurens P. Bosman, Monica Chivulescu, Crystal Tichnell, Brittney Murray, Harikrishna Tandri, Rafik Tadros, Lena Rivard, Maarten P. Van Den Berg, Katja Zeppenfeld, Arthur A.M. Wilde, Giulio Pompilio, Corrado Carbucicchio, Antonio Dello Russo, Michela Casella, Anneli Svensson, Corinna B. BrunckhorstJ. Peter Van Tintelen, Pyotr G. Platonov, Kristina H. Haugaa, Firat Duru, Anneline S.J.M. Te Riele, Paul Khairy, Claudio Tondo, Hugh Calkins, Cynthia A. James, Ardan M. Saguner, Julia Cadrin-Tourigny*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

16 Citations (Scopus)
21 Downloads (Pure)


Background: A novel risk calculator based on clinical characteristics and noninvasive tests that predicts the onset of clinical sustained ventricular arrhythmias (VA) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) has been proposed and validated by recent studies. It remains unknown whether programmed ventricular stimulation (PVS) provides additional prognostic value. Methods: All patients with a definite ARVC diagnosis, no history of sustained VAs at diagnosis, and PVS performed at baseline were extracted from 6 international ARVC registries. The calculator-predicted risk for sustained VA (sustained or implantable cardioverter defibrillator treated ventricular tachycardia [VT] or fibrillation, [aborted] sudden cardiac arrest) was assessed in all patients. Independent and combined performance of the risk calculator and PVS on sustained VA were assessed during a 5-year follow-up period. Results: Two hundred eighty-eight patients (41.0±14.5 years, 55.9% male, right ventricular ejection fraction 42.5±11.1%) were enrolled. At PVS, 137 (47.6%) patients had inducible ventricular tachycardia. During a median of 5.31 [2.89-10.17] years of follow-up, 83 (60.6%) patients with a positive PVS and 37 (24.5%) with a negative PVS experienced sustained VA (P<0.001). Inducible ventricular tachycardia predicted clinical sustained VA during the 5-year follow-up and remained an independent predictor after accounting for the calculator-predicted risk (HR, 2.52 [1.58-4.02]; P<0.001). Compared with ARVC risk calculator predictions in isolation (C-statistic 0.72), addition of PVS inducibility showed improved prediction of VA events (C-statistic 0.75; log-likelihood ratio for nested models, P<0.001). PVS inducibility had a 76% [67-84] sensitivity and 68% [61-74] specificity, corresponding to log-likelihood ratios of 2.3 and 0.36 for inducible (likelihood ratio+) and noninducible (likelihood ratio-) patients, respectively. In patients with a ARVC risk calculator-predicted risk of clinical VA events <25% during 5 years (ie, low/intermediate subgroup), PVS had a 92.6% negative predictive value. Conclusions: PVS significantly improved risk stratification above and beyond the calculator-predicted risk of VA in a primary prevention cohort of patients with ARVC, mainly for patients considered to be at low and intermediate risk by the clinical risk calculator.

Original languageEnglish
Pages (from-to)1434-1443
Number of pages10
Issue number19
Publication statusPublished - 8-Nov-2022


  • arrhythmogenic right ventricular cardiomyopathy
  • defibrillator, implantable
  • electrophysiological techniques, cardiac
  • risk assessment
  • sudden cardiac death


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