Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy: external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator-Reference-Cited by-同舟云学术

Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy: external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator

Published:2022-06-29 Issue:32 Volume:43 Page:3041-3052
ISSN:0195-668X
Container-title:European Heart Journal
language:en
Short-container-title:

Author:

Jordà Paloma¹²³^ORCID,Bosman Laurens P⁴,Gasperetti Alessio⁵,Mazzanti Andrea⁶⁷⁸,Gourraud Jean Baptiste⁹,Davies Brianna¹⁰,Frederiksen Tanja Charlotte¹¹¹²,Weidmann Zoraida Moreno¹³,Di Marco Andrea¹⁴,Roberts Jason D¹⁵¹⁶¹⁷,MacIntyre Ciorsti¹⁸,Seifer Colette¹⁹,Delinière Antoine²⁰^ORCID,Alqarawi Wael²¹,Kukavica Deni⁶⁷⁸^ORCID,Minois Damien⁹^ORCID,Trancuccio Alessandro⁶⁷⁸^ORCID,Arnaud Marine⁹^ORCID,Targetti Mattia²²,Martino Annamaria²³,Oliviero Giada²³,Pipilas Daniel C²⁴,Carbucicchio Corrado²⁵,Compagnucci Paolo²⁶,Dello Russo Antonio²⁶,Olivotto Iacopo²²,Calò Leonardo²³^ORCID,Lubitz Steven A²⁴,Cutler Michael J²⁷,Chevalier Philippe²⁰^ORCID,Arbelo Elena²³²⁸²⁹^ORCID,Priori Silvia Giuliana⁶⁷⁸^ORCID,Healey Jeffrey S¹⁵¹⁶,Calkins Hugh⁵^ORCID,Casella Michela³⁰^ORCID,Jensen Henrik Kjærulf¹¹¹²,Tondo Claudio³¹³²,Tadros Rafik¹^ORCID,James Cynthia A⁵,Krahn Andrew D¹⁰,Cadrin-Tourigny Julia¹^ORCID

Affiliation:

1. Cardiovascular Genetics Center, Montreal Heart Institute, Université de Montréal , Montréal, QC , Canada

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

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

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

5. Division of Cardiology, Department of Medicine, Johns Hopkins Hospital , Baltimore, MD , USA

6. Department of Molecular Medicine, University of Pavia , Pavia , Italy

7. Department of Molecular Cardiology, IRCCS Istituti Clinici Scientifici Maugeri , Pavia , Italy

8. Department of Molecular Cardiology, Centro Nacional de Investigaciones Cardiovasculares , Madrid , Spain

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

10. Centre for Cardiovascular Innovation, Division of Cardiology, University of British Columbia , Vancouver, BC , Canada

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

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

13. Department of Cardiology, Hospital de la Santa Creu i Sant Pau, IIB Sant Pau, Universitat Autònoma de Barcelona , CIBERCV, Barcelona , Spain

14. Arrhythmia Unit, Department of Cardiology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat , Barcelona , Spain

15. Population Health Research Institute, McMaster University , Hamilton, ON , Canada

16. Division of Cardiology, Hamilton Health Sciences , Hamilton, ON , Canada

17. Section of Cardiac Electrophysiology, Division of Cardiology, Department of Medicine, Western University , London, ON , Canada

18. Cardiac Electrophysiology Service, Quenn Elisabeth II Health Sciences Center, Dalhousie University , Halifax, NS , Canada

19. St-Boniface Hospital, University of Manitoba , Winnipeg, MB , Canada

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

21. Cardiac Electrophysiology Service, Ottawa Heart Institute, University of Ottawa , Ottawa, ON , Canada

22. Cardiomyopathy Unit, Department of Cardiology, Careggi University Hospital , Florence , Italy

23. Department of Cardiology, Policlinico Casilino , Rome , Italy

24. Cardiovascular Research Center and Cardiac Arrhythmia Service, Massachusetts General Hospital , Boston, MA , USA

25. Department of Clinical Electrophysiology and Cardiac Pacing Centro Cardiologico Monzino , IRCCSC, Milan , Italy

26. Department of Biomedical Sciences and Public Health, Cardiology and Arrhythmology Clinic, University Hospital Umberto I-Salesi-Lancisi, Marche Polytechnic University , Ancona , Italy

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

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

29. European Reference Network for rare, low prevalence and complex diseases of the heart - ERN GUARD-Heart

30. Department of Clinical, Special and Dental Sciences, Cardiology and Arrhythmology Clinic, University Hospital Umberto I-Salesi-Lancisi, MarchePolytechnic University , Ancona , Italy

31. Department of Clinical Electrophysiology and Cardiac Pacing Centro Cardiologico Monzino, IRCCSC , Milan , Italy

32. Department of Biomedical, Surgical and Dentistry Sciences, University of Milan , Milan , Italy

Abstract

Abstract Aims Arrhythmogenic right ventricular cardiomyopathy (ARVC) causes ventricular arrhythmias (VAs) and sudden cardiac death (SCD). In 2019, a risk prediction model that estimates the 5-year risk of incident VAs in ARVC was developed (ARVCrisk.com). This study aimed to externally validate this prediction model in a large international multicentre cohort and to compare its performance with the risk factor approach recommended for implantable cardioverter-defibrillator (ICD) use by published guidelines and expert consensus. Methods and results In a retrospective cohort of 429 individuals from 29 centres in North America and Europe, 103 (24%) experienced sustained VA during a median follow-up of 5.02 (2.05–7.90) years following diagnosis of ARVC. External validation yielded good discrimination [C-index of 0.70 (95% confidence interval-CI 0.65–0.75)] and calibration slope of 1.01 (95% CI 0.99–1.03). Compared with the three published consensus-based decision algorithms for ICD use in ARVC (Heart Rhythm Society consensus on arrhythmogenic cardiomyopathy, International Task Force consensus statement on the treatment of ARVC, and American Heart Association guidelines for VA and SCD), the risk calculator performed better with a superior net clinical benefit below risk threshold of 35%. Conclusion Using a large independent cohort of patients, this study shows that the ARVC risk model provides good prognostic information and outperforms other published decision algorithms for ICD use. These findings support the use of the model to facilitate shared decision making regarding ICD implantation in the primary prevention of SCD in ARVC.

Funder

Daniel Bravo Foundation

Spanish Society of Cardiology Magda Heras

Wilton W. Webster

Heart Rhythm Society

The Johns Hopkins

Leonie-Wild Foundation

Leyla Erkan Family Fund

ARVD Research

Hugh Calkins

Marvin H. Weiner

Jacqueline J. Bernstein Cardiac Arrhythmia Center