Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy : external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator
Jordà, Paloma (Institut d'Investigacions Biomèdiques August Pi i Sunyer)
Bosman, Laurens P. (University Medical Center Utrech)
Gasperetti, Alessio (Johns Hopkins Hospital)
Mazzanti, Andrea (Centro Nacional de Investigaciones Cardiovasculares. Department of Molecular Cardiology)
Gourraud, Jean Baptiste (Centre Hospitalier Universitaire Nantes. Department of Cardiology)
Davies, Brianna (Centre for Cardiovascular Innovation. Division of Cardiology. University of British Columbia)
Frederiksen, Tanja Charlotte (Aarhus University. Department of Clinical Medicine Health)
Moreno Weidmann, Zoraida (Institut d'Investigació Biomèdica Sant Pau)
Di Marco, Andrea (Hospital Universitari de Bellvitge)
Roberts, Jason D. (Western University. Section of Cardiac Electrophysiology)
MacIntyre, Ciorsti (Cardiac Electrophysiology Service. Quenn Elisabeth II Health Sciences Center. Dalhousie University)
Seifer, Colette (St-Boniface Hospital. University of Manitoba)
Delinière, Antoine (National Reference Center for Inherited Arrhythmias of Lyon. Louis Pradel Cardiovascular Hospital. Hospices Civils de Lyon)
Alqarawi, Wael (University of Ottawa (Ottawa, Canadà). Cardiac Electrophysiology Service)
Kukavica, Deni (Centro Nacional de Investigaciones Cardiovasculares. Department of Molecular Cardiology)
Minois, Damien (Centre Hospitalier Universitaire Nantes)
Trancuccio, Alessandro (Centro Nacional de Investigaciones Cardiovasculares Carlos III (CNIC))
Arnaud, Marine (Centre Hospitalier Universitaire Nantes)
Targetti, Mattia (Careggi University Hospital (Florència, Itàlia))
Martino, Annamaria (Policlinico Casilino (Roma, Itàlia). Department of Cardiology)
Oliviero, Giada (Policlinico Casilino (Roma, Itàlia). Department of Cardiology)
Pipilas, Daniel C. (Massachusetts General Hospital (Boston))
Carbucicchio, Corrado (Centro Cardiologico Monzino (Milà, Itàlia))
Compagnucci, Paolo (University Hospital Umberto I-Salesi-Lancisi. Marche Polytechnic University)
Dello Russo, Antonio (University Hospital Umberto I-Salesi-Lancisi. Marche Polytechnic University)
Olivotto, Iacopo (Careggi University Hospital (Florència, Itàlia))
Calò, Leonardo (Policlinico Casilino (Roma, Itàlia). Department of Cardiology)
Lubitz, Steven A. (Massachusetts General Hospital (Boston))
Cutler, Michael J. (Intermountain Medical Center Heart Institute)
Chevalier, Philippe (National Reference Center for Inherited Arrhythmias of Lyon. Louis Pradel Cardiovascular Hospital. Hospices Civils de Lyon)
Arbelo, Elena (European Reference Network for rare. low prevalence and complex diseases of the heart. ERN GUARD-Heart)
Priori, Silvia (Centro Nacional de Investigaciones Cardiovasculares. Department of Molecular Cardiology)
Healey, Jeffrey S. (Hamilton Health Sciences. Division of Cardiology)
Calkins, Hugh (Johns Hopkins Hospital)
Casella, Michela (University Hospital Umberto I-Salesi-Lancisi. MarchePolytechnic University)
Jensen, Henrik Kjærulf (Aarhus University. Department of Clinical Medicine Health)
Tondo, Claudio (University of Milan. Department of Biomedical, Surgical and Dentistry Sciences)
Tadros, Rafik (Montreal Heart Institute. Cardiovascular Genetics Center)
James, Cynthia (Johns Hopkins Hospital. Division of Cardiology)
Krahn, Andrew D. (University of British Columbia. Centre for Cardiovascular Innovation)
Cadrin-Tourigny, Julia (Montreal Heart Institute. Cardiovascular Genetics Center)
Universitat Autònoma de Barcelona

Data:	2022
Resum:	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.
Drets:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, sempre que no sigui amb finalitats comercials, i sempre que es reconegui l'autoria de l'obra original.
Llengua:	Anglès
Document:	Article ; recerca ; Versió publicada
Matèria:	Arrhythmogenic right ventricular cardiomyopathy ; Implantable cardioverter-defibrillator ; Sudden cardiac death ; Ventricular arrhythmias ; Risk stratification ; Genetic cardiomyopathies
Publicat a:	European heart journal, Vol. 43 Núm. 32 (August 2022) , p. 3041-3052, ISSN 1522-9645

DOI: 10.1093/eurheartj/ehac289
PMID: 35766180

12 p, 1.5 MB

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