TY - JOUR
T1 - Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy
T2 - external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator
AU - Jordà, Paloma
AU - Bosman, Laurens P.
AU - Gasperetti, Alessio
AU - Mazzanti, Andrea
AU - Gourraud, Jean Baptiste
AU - Davies, Brianna
AU - Frederiksen, Tanja Charlotte
AU - Weidmann, Zoraida Moreno
AU - Di Marco, Andrea
AU - Roberts, Jason D.
AU - Macintyre, Ciorsti
AU - Seifer, Colette
AU - Delinière, Antoine
AU - Alqarawi, Wael
AU - Kukavica, Deni
AU - Minois, Damien
AU - Trancuccio, Alessandro
AU - Arnaud, Marine
AU - Targetti, Mattia
AU - Martino, Annamaria
AU - Oliviero, Giada
AU - Pipilas, Daniel C.
AU - Carbucicchio, Corrado
AU - Compagnucci, Paolo
AU - Dello Russo, Antonio
AU - Olivotto, Iacopo
AU - Calò, Leonardo
AU - Lubitz, Steven A.
AU - Cutler, Michael J.
AU - Chevalier, Philippe
AU - Arbelo, Elena
AU - Priori, Silvia Giuliana
AU - Healey, Jeffrey S.
AU - Calkins, Hugh
AU - Casella, Michela
AU - Jensen, Henrik Kjærulf
AU - Tondo, Claudio
AU - Tadros, Rafik
AU - James, Cynthia A.
AU - Krahn, Andrew D.
AU - Cadrin-Tourigny, Julia
N1 - Publisher Copyright:
© 2022 The Author(s). Published by Oxford University Press on behalf of European Society of Cardiology.
PY - 2022/8/21
Y1 - 2022/8/21
N2 - 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.
AB - 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.
KW - Arrhythmogenic right ventricular cardiomyopathy
KW - Genetic cardiomyopathies
KW - Implantable cardioverter-defibrillator
KW - Risk stratification
KW - Sudden cardiac death
KW - Ventricular arrhythmias
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U2 - 10.1093/eurheartj/ehac289
DO - 10.1093/eurheartj/ehac289
M3 - Article
C2 - 35766180
AN - SCOPUS:85141202073
SN - 0195-668X
VL - 43
SP - 3041
EP - 3052
JO - European heart journal
JF - European heart journal
IS - 32
ER -