Importance of genotype for risk stratification in arrhythmogenic right ventricular cardiomyopathy using the 2019 ARVC risk calculator
European Heart Journal
Abstract
To study the impact of genotype on the performance of the 2019 risk model for arrhythmogenic right ventricular cardiomyopathy (ARVC).
The study cohort comprised 554 patients with a definite diagnosis of ARVC and no history of sustained ventricular arrhythmia (VA). During a median follow-up of 6.0 (3.1,12.5) years, 100 patients (18%) experienced the primary VA outcome (sustained ventricular tachycardia, appropriate implantable cardioverter defibrillator intervention, aborted sudden cardiac arrest, or sudden cardiac death) corresponding to an annual event rate of 2.6% [95% confidence interval (CI) 1.9–3.3]. Risk estimates for VA using the 2019 ARVC risk model showed reasonable discriminative ability but with overestimation of risk. The ARVC risk model was compared in four gene groups:
The 2019 ARVC risk model performs reasonably well in gene-positive ARVC (particularly for
Contributors
Luis Rocha Lopes
Author
Konstantinos Savvatis
Author
Barts Heart Centre London , United Kingdom of Great Britain & Northern Ireland
Davide Stolfo
Author
Marco Merlo
Author
Javier Limeres Freire
Author
Alexandros Protonotarios
Author
University College London London , United Kingdom of Great Britain & Northern Ireland
Riccardo Bariani
Author
Chiara Cappelletto
Author
Menelaos Pavlou
Author
Toru Kubo
Author
Alba García-García
Author
Alberto Cipriani
Author
Ioannis Protonotarios
Author
Adrian Rivas
Author
Regitze Wittenberg
Author
Maddalena Graziosi
Author
Zafeirenia Xylouri
Author
Loizos Antoniades
Author
José M Larrañaga-Moreira
Author
Rudy Celeghin
Author
Kalliopi Pilichou
Author
Athanasios Bakalakos
Author
Jens Mogensen
Author
Pablo Garcia-Pavia
Author
Karim Wahbi
Author
Aris Anastasakis
Author
Adalena Tsatsopoulou
Author
Esther Zorio
Author
Juan R Gimeno
Author
Jose Manuel Garcia-Pinilla
Author
Petros Syrris
Author
Barbara Bauce
Author
Perry M Elliott
Author
University College London London , United Kingdom of Great Britain & Northern Ireland
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