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Evolution and triggers of defibrillator shocks in patients with arrhythmogenic right ventricular cardiomyopathy
  1. Nadine Molitor1,
  2. Daniel Hofer1,
  3. Tolga Çimen1,
  4. Alessio Gasperetti1,2,
  5. Deniz Akdis1,3,
  6. Sarah Costa1,
  7. Rolf Jenni1,
  8. Alexander Breitenstein1,
  9. Thomas Wolber1,4,
  10. Stephan Winnik1,
  11. Siv Fokstuen1,5,
  12. Guan Fu1,
  13. Argelia Medeiros-Domingo6,
  14. Frank Ruschitzka1,
  15. Corinna Brunckhorst1,
  16. Firat Duru1,4,
  17. Ardan M Saguner1
  1. 1Cardiology, Department of Cardiology, University Heart Center, University Hospital Zurich, Zurich, Switzerland
  2. 2Department of Medicine, Division of Cardiology, Johns Hopkins University, Baltimore, Maryland, US
  3. 3Division of Cardiology, GZO - Regional Health Center, Wetzikon, Switzerland
  4. 4Center for Integrative Human Physiology (ZIHP), University of Zurich, Zurich, Switzerland
  5. 5Genetic Medicine division, Diagnostic Department, Hôpitaux Universitaires de Genève, Genève, Switzerland
  6. 6Swiss DNAlysis Dübendorf, Zurich, Switzerland
  1. Correspondence to Dr. med. Nadine Molitor, Cardiology, University Hospital Zurich, Zurich, Switzerland; nadine.molitor{at}; Dr. med. Ardan M Saguner; ardan.saguner{at}


Introduction Implantable cardioverter-defibrillators (ICDs) can prevent sudden cardiac death due to ventricular arrhythmias in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). The aim of our study was to assess the cumulative burden, evolution and potential triggers of appropriate ICD shocks during long-term follow-up, which may help to reduce and further refine individual arrhythmic risk in this challenging disease.

Methods This retrospective cohort study included 53 patients with definite ARVC according to the 2010 Task Force Criteria from the multicentre Swiss ARVC Registry with an implanted ICD for primary or secondary prevention. Follow-up was conducted by assessing all available patient records from patient visits, hospitalisations, blood samples, genetic analysis, as well as device interrogation and tracings.

Results Fifty-three patients (male 71.7%, mean age 43±2.2 years, genotype positive 58.5%) were analysed during a median follow-up of 7.9 (IQR 10) years. In 29 (54.7%) patients, 177 appropriate ICD shocks associated with 71 shock episodes occurred. Median time to first appropriate ICD shock was 2.8 (IQR 3.6) years. Long-term risk of shocks remained high throughout long-term follow-up. Shock episodes occurred mainly during daytime (91.5%, n=65) and without seasonal preference. We identified potentially reversible triggers in 56 of 71 (78.9%) appropriate shock episodes, the main triggers representing physical activity, inflammation and hypokalaemia.

Conclusion The long-term risk of appropriate ICD shocks in patients with ARVC remains high during long-term follow-up. Ventricular arrhythmias occur more often during daytime, without seasonal preference. Reversible triggers are frequent with the most common triggers for appropriate ICD shocks being physical activity, inflammation and hypokalaemia in this patient population.

  • tachycardia, ventricular
  • ventricular fibrillation
  • cardiomyopathies
  • defibrillators, implantable

Data availability statement

Data are available on reasonable request. On urgent request and associated need, our data are available, while our upmost intention is to protect our patient’s privacy.

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Data availability statement

Data are available on reasonable request. On urgent request and associated need, our data are available, while our upmost intention is to protect our patient’s privacy.

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  • NM and DH contributed equally.

  • Correction notice This article has been corrected since it was first published online. A second affiliation has been added for author Deniz Akdis.

  • Contributors Conceptualisation: AMS. Data curation: DH and NM. Analysis of data: NM, AMS, DH. Drafting the Manuscript: NM. Review and editing: TC, AG, DA, SC, RJ, AB, TW, SW, SF, GF, AM-D, FR, CB, FD, and AMS. Guarantor: AMS.

  • Funding The Zurich ARVC Program is supported by grants from the Georg and Bertha Schwyzer-Winiker Foundation, Baugarten Foundation, Swiss National Science Foundation (grant no: 160327), Swiss Heart Foundation (grant no: FF17019 and FF21073) and USZ Foundation (Dr Wild Grant).

  • Competing interests AMS received educational grants through his institution from Abbott, Bayer Healthcare, Biosense Webster, Biotronik, Boston Scientific, BMS/Pfizer, Medtronic and Novartis, and speaker/consultant fees from Bayer Healthcare, Biotronik, BMS/Pfizer, Daiichi-Sankyo, Medtronic, Novartis and Stride Bio. DH reports educational grants, consultant or speaker fees and fellowship support from Abbott, Bayer, Biotronik, Biosense Webster, Boston Scientific, Medtronic, Novartis, Pfizer and Spectranetics/Philips. AB has received consultant and/or speaker fees from Abbott, Bayer Healthcare, Biosense Webster, Biotronik, Boston Scientific, Bristol-Myers Squibb, Cook Medical, Daiichi Sankyo, Medtronic, Pfizer, Spectranetics/Philipps and ZOLL. AM-D is the founder of SwissDNAlysis. FR has not received personal payments by pharmaceutical companies or device manufacturers in the last 3 years (remuneration for the time spent in activities, such as participation as steering committee member of clinical trials and member of the Pfizer Research Award selection committee in Switzerland were made directly to the University of Zurich). The Department of Cardiology (University Hospital of Zurich/University of Zurich) reports research, educational and/or travel grants from Abbott, Amgen, Astra Zeneca, Berlin Heart, B. Braun, Biosense Webster, Biosensors Europe AG, Biotronik, BMS, Boehringer Ingelheim, Boston Scientific, Bracco, Cardinal Health Switzerland, Corteria, Daiichi, Diatools AG, Edwards Lifesciences, Guidant Europe NV (BS), Hamilton Health Sciences, Kaneka Corporation, Kantar, Labormedizinisches Zentrum, Medtronic, MSD, Mundipharma Medical Company, Novartis, Novo Nordisk, Orion, Pfizer, Quintiles Switzerland Sarl, Roche Diagnostics, Sahajanand IN, Sanofi, Sarstedt AG, Servier, SIS Medical, SSS International Clinical Research, Terumo Deutschland, Trama Solutions, V-Wave, Vascular Medical, Vifor, Wissens Plus and ZOLL. The research and educational grants do not impact on FR's personal remuneration. The other authors have nothing to disclose related to the content of this article.

  • Patient and public involvement Patients and/or the public were not involved in the design, conduct, reporting or dissemination plans of this research.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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