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00925njm 22002777a 4500 dc Jordà, Paloma author Bosman, Laurens P author Gasperetti, Alessio author Mazzanti, Andrea author Gourraud, Jean Baptiste author Davies, Brianna author Frederiksen, Tanja Charlotte author Weidmann, Zoraida Moreno author Di Marco, Andrea author Roberts, Jason D author MacIntyre, Ciorsti author Seifer, Colette author Delinière, Antoine author Alqarawi, Wael author Kukavica, Deni author Minois, Damien author Trancuccio, Alessandro author Arnaud, Marine author Targetti, Mattia author Martino, Annamaria author Oliviero, Giada author Pipilas, Daniel C author Carbucicchio, Corrado author Compagnucci, Paolo author Dello Russo, Antonio author Olivotto, Iacopo author Calò, Leonardo author Lubitz, Steven A author Cutler, Michael J author Chevalier, Philippe author Arbelo, Elena author Priori, Silvia G. author Healey, Jeffrey S author Calkins, Hugh author Casella, Michela author Jensen, Henrik Kjærulf author Tondo, Claudio author Tadros, Rafik author James, Cynthia A author Krahn, Andrew D author Cadrin-Tourigny, Julia author 2022-08-21 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. 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%. 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. Eur Heart J. 2022 Aug 21;43(32):3041-3052 10.1093/eurheartj/ehac289 1522-9645 European heart journal 35766180 http://hdl.handle.net/20.500.12105/15742 Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy: external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator.