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

Author:

Carrick Richard T1ORCID,De Marco Corrado2ORCID,Gasperetti Alessio13ORCID,Bosman Laurens P34ORCID,Gourraud Jean-Baptiste45ORCID,Trancuccio Alessandro6ORCID,Mazzanti Andrea6ORCID,Murray Brittney1ORCID,Pendleton Catherine1,Tichnell Crystal1,Tandri Harikrishna17,Zeppenfeld Katja8ORCID,Wilde Arthur A M49,Davies Brianna10ORCID,Seifer Colette11,Roberts Jason D1213,Healey Jeff S12,MacIntyre Ciorsti1415,Alqarawi Wael1617ORCID,Tadros Rafik2,Cutler Michael J18,Targetti Mattia19,Calò Leonardo20,Vitali Francesco21,Bertini Matteo21ORCID,Compagnucci Paolo22ORCID,Casella Michela22,Dello Russo Antonio22,Cappelletto Chiara42324ORCID,De Luca Antonio423,Stolfo Davide42324ORCID,Duru Firat25ORCID,Jensen Henrik K42627,Svensson Anneli2829,Dahlberg Pia30,Hasselberg Nina E31,Di Marco Andrea3233ORCID,Jordà Paloma234,Arbelo Elena4343536ORCID,Moreno Weidmann Zoraida37,Borowiec Karolina3839,Delinière Antoine44041ORCID,Biernacka Elżbieta K3839,van Tintelen J Peter442,Platonov Pyotr G43,Olivotto Iacopo19,Saguner Ardan M25ORCID,Haugaa Kristina H31,Cox Moniek44,Tondo Claudio4546,Merlo Marco423ORCID,Krahn Andrew D10ORCID,te Riele Anneline S J M34ORCID,Wu Katherine C1,Calkins Hugh1ORCID,James Cynthia A1ORCID,Cadrin-Tourigny Julia2ORCID

Affiliation:

1. Heart and Vascular Institute, Johns Hopkins University , Baltimore, MD , USA

2. Cardiovascular Genetics Centre, Montreal Heart Institute, Université de Montréal , 5000 rue Bélanger, Montréal, Québec H1T 1C8 , Canada

3. Division of Heart and Lungs, Department of Cardiology, University Medical Center Utrecht, Utrecht University , Utrecht , The Netherlands

4. Member of the European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN GUARD-Heart)

5. Department of Cardiology, Centre Hospitalier Universitaire Nantes , Nantes , France

6. Molecular Cardiology, Istituti Clinici Scientifici Maugeri (IRCCS) , Pavia , Italy

7. Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA

8. Department of Cardiology, Leiden University Medical Center , Leiden , The Netherlands

9. Amsterdam UMC, Heart Center Department of Cardiology, Amsterdam Cardiovascular Sciences, Heart Failure and Arrhythmias, University of Amsterdam , Amsterdam , The Netherlands

10. Center for Cardiac Innovation, Division of Cardiology, University of British Columbia , Vancouver, British Columbia , Canada

11. St.Boniface Hospital, University of Manitoba , Winnipeg, Manitoba , Canada

12. Hamilton Health Sciences, Population Health Research Institute, McMaster University , Hamilton, Ontario , Canada

13. Section of Cardiac Electrophysiology, Division of Cardiology, Department of Medicine, Western University , London, Ontario , Canada

14. Queen Elizabeth II Health Sciences Centre, Dalhousie University , Halifax, Nova Scotia , Canada

15. Department of Medicine, Mayo Clinic , Rochester, Minnesota , USA

16. Department of Cardiac Sciences, College of Medicine, King Saudi University , Riyadh , Saudi Arabia

17. Division of Cardiology, University of Ottawa Heart Institute, University of Ottawa , Ottawa , Canada

18. Intermountain Medical Center, Intermountain Medical Center Heart Institute , Murray, UT , USA

19. Cardiomyopathy Unit, Careggi Hospital and Meyer Children’s Hospital IRCCS , Florence , Italy

20. Division of Cardiology, Policlinico Casilino , Rome , Italy

21. Cardiology Unit, Sant’Anna University Hospital, University of Ferrara , Ferrara , Italy

22. Cardiology and Arrhythmology Clinic, University Hospital Ospedali Riuniti , Ancona , Italy

23. Division of Cardiology, Cardiothoracovascular Department, Azienda Sanitaria Universitaria Giuliano Isontina and University of Trieste , Trieste , Italy

24. Division of Cardiology, Department of Medicine, Karolinska Institutet, Solna , Stockholm , Sweden

25. Department of Cardiology, University Heart Center Zurich , Zurich , Switzerland

26. Department of Cardiology, Aarhus University Hospital , Aarhus N , Denmark

27. Department of Clinical Medicine, Health, Aarhus University , Aarhus N , Denmark

28. Department of Cardiology, Linköping University Hospital , Linköping , Sweden

29. Department of Health, Medicine and Caring Sciences, Linköping University , Linköping , Sweden

30. Department of Cardiology, Sahlgrenska University Hospital , Gothenburg , Sweden

31. ProCardio Center for Innovation, Department of Cardiology, Oslo University Hospital, Rikshospitalet, University of Oslo , Oslo , Norway

32. Arrhythmia Unit, Department ofCardiology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat , Barcelona , Spain

33. BioHeartCardiovascular Diseases Research Group, Bellvitge Biomedical Research Institute (IDIBELL), L'Hospitalet de Llobregat , Barcelona , Spain

34. Arrhythmia Section, Department of Cardiology, Hospital Clínic, Universitat de Barcelona , Barcelona , Spain

35. Institut d'Investigació August Pi i Sunyer (IDIBAPS), Barcelona, Spain

36. Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Madrid, Spain

37. Arrhythmia Unit, Department of Cardiology, Hospital Universitari Sant Pau , Barcelona , Spain

38. Department of Congenital Heart Diseases, Cardinal Wyszynski National Institute of Cardiology , Warsaw , Poland

39. Outpatient Department of Genetic Arrhythmias, Cardinal Wyszynski National Institute of Cardiology , Warsaw , Poland

40. Department of Cardiology, National Reference Center for Inherited Arrhythmias of Lyon, Louis Pradel Cardiovascular Hospital, Hospices Civils de Lyon , Lyon , France

41. University of Lyon, Claude Bernard Lyon 1 University, MeLiS, CNRS UMR 5284, INSERM U1314, Institut NeuroMyoGène, Lyon, France

42. Department of Genetics, University Medical Center Utrecht, Utrecht University , Utrecht , The Netherlands

43. Department of Cardiology, Clinical Sciences, Lund University , Lund , Sweden

44. Department of Cardiology, University Medical Center Groningen , Groningen , The Netherlands

45. Department of Clinical Electrophysiology and Cardiac Pacing, Centro Cardiologico Monzino, IRCCS, University of Milan , Milan , Italy

46. Department of Biomedical, Surgical and Dental Sciences, University of Milan, Milan, Italy

Abstract

Abstract 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.

Funder

Leonie-Wild Foundation

Leyla Erkan Family Fund

ARVD

Hugh Calkins

Bernstein Cardiac Arrhythmia Center

Chiaramonte Private Foundation

Robin Shah ARVD Fund

Bogle Foundation

Campanella Family

Harrison Family

Peter French Memorial Foundation

Wilmerding Endowments

NIH

Philippa and Marvin Carsley Cardiology Research Chair

Montreal Heart Institute Foundation

FRQS

Norwegian Research Council

ZonMW

Dutch Heart Association

Netherlands Cardiovascular Research Initiative

Dutch Heart Foundation

Netherlands Arrhythmogenic Cardiomyopathy

ACM

Netherlands Heart Institute

Zurich ARVC Program

Georg and Bertha Schwyzer-Winiker Foundation

Baugarten Foundation

Swiss National Science Foundation

Swiss Heart Foundation

Spanish Society of Cardiology

Swedish Heart Lung and ALF foundations

Publisher

Oxford University Press (OUP)

Subject

Cardiology and Cardiovascular Medicine

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