Implantable cardioverter defibrillator use in arrhythmogenic right ventricular cardiomyopathy in North America and Europe

Richard T Carrick, Corrado De Marco, Alessio Gasperetti, Laurens P Bosman, Jean-Baptiste Gourraud, Alessandro Trancuccio, Andrea Mazzanti, Brittney Murray, Catherine Pendleton, Crystal Tichnell, Harikrishna Tandri, Katja Zeppenfeld, Arthur A M Wilde, Brianna Davies, Colette Seifer, Jason D Roberts, Jeff S Healey, Ciorsti MacIntyre, Wael Alqarawi, Rafik TadrosMichael J Cutler, Mattia Targetti, Leonardo Calò, Francesco Vitali, Matteo Bertini, Paolo Compagnucci, Michela Casella, Antonio Dello Russo, Chiara Cappelletto, Antonio De Luca, Davide Stolfo, Firat Duru, Henrik K Jensen, Anneli Svensson, Pia Dahlberg, Nina E Hasselberg, Andrea Di Marco, Paloma Jordà, Elena Arbelo, Zoraida Moreno Weidmann, Karolina Borowiec, Antoine Delinière, Elżbieta K Biernacka, J Peter van Tintelen, Pyotr G Platonov, Iacopo Olivotto, Ardan M Saguner, Kristina H Haugaa, Moniek Cox, Claudio Tondo, Marco Merlo, Andrew D Krahn, Anneline S J M Te Riele, Katherine C Wu, Hugh Calkins, Cynthia A James, Julia Cadrin-Tourigny*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review


BACKGROUND AND AIMS: Implantable cardioverter-defibrillators (ICDs) are critical for preventing sudden cardiac death (SCD) in arrhythmogenic right ventricular cardiomyopathy (ARVC). This study aims to identify cross-continental differences in utilization of primary prevention ICDs and survival free from sustained ventricular arrhythmia (VA) in ARVC.

METHODS: This was a retrospective analysis of ARVC patients without prior VA enrolled in clinical registries from 11 countries throughout Europe and North America. Patients were classified according to whether they received treatment in North America or Europe and were further stratified by baseline predicted VA risk into low- (<10%/5 years), intermediate- (10%-25%/5 years), and high-risk (>25%/5 years) groups. Differences in ICD implantation and survival free from sustained VA events (including appropriate ICD therapy) were assessed.

RESULTS: One thousand ninety-eight patients were followed for a median of 5.1 years; 554 (50.5%) received a primary prevention ICD, and 286 (26.0%) experienced a first VA event. After adjusting for baseline risk factors, North Americans were more than three times as likely to receive ICDs {hazard ratio (HR) 3.1 [95% confidence interval (CI) 2.5, 3.8]} but had only mildly increased risk for incident sustained VA [HR 1.4 (95% CI 1.1, 1.8)]. North Americans without ICDs were at higher risk for incident sustained VA [HR 2.1 (95% CI 1.3, 3.4)] than Europeans.

CONCLUSIONS: North American ARVC patients were substantially more likely than Europeans to receive primary prevention ICDs across all arrhythmic risk strata. A lower rate of ICD implantation in Europe was not associated with a higher rate of VA events in those without ICDs.

Original languageEnglish
Pages (from-to)538-548
Number of pages11
JournalEuropean heart journal
Issue number7
Early online date9 Jan 2024
Publication statusPublished - 14 Feb 2024


  • Arrhythmogenic right ventricular cardiomyopathy
  • Implantable cardioverter-defibrillator
  • Primary prevention
  • Sudden cardiac death
  • Ventricular arrhythmia


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