RT Journal Article
T1 Importance of genotype for risk stratification in arrhythmogenic right ventricular cardiomyopathy using the 2019 ARVC risk calculator.
A1 Protonotarios, Alexandros
A1 Bariani, Riccardo
A1 Cappelletto, Chiara
A1 Pavlou, Menelaos
A1 García-García, Alba
A1 Cipriani, Alberto
A1 Protonotarios, Ioannis
A1 Rivas, Adrian
A1 Wittenberg, Regitze
A1 Graziosi, Maddalena
A1 Xylouri, Zafeirenia
A1 Larrañaga-Moreira, José M
A1 de Luca, Antonio
A1 Celeghin, Rudy
A1 Pilichou, Kalliopi
A1 Bakalakos, Athanasios
A1 Lopes, Luis Rocha
A1 Savvatis, Konstantinos
A1 Stolfo, Davide
A1 Dal Ferro, Matteo
A1 Merlo, Marco
A1 Basso, Cristina
A1 Freire, Javier Limeres
A1 Rodriguez-Palomares, Jose F
A1 Kubo, Toru
A1 Ripoll-Vera, Tomas
A1 Barriales-Villa, Roberto
A1 Antoniades, Loizos
A1 Mogensen, Jens
A1 Garcia-Pavia, Pablo
A1 Wahbi, Karim
A1 Biagini, Elena
A1 Anastasakis, Aris
A1 Tsatsopoulou, Adalena
A1 Zorio, Esther
A1 Gimeno, Juan R
A1 Garcia-Pinilla, Jose Manuel
A1 Syrris, Petros
A1 Sinagra, Gianfranco
A1 Bauce, Barbara
A1 Elliott, Perry M
K1 Arrhythmogenic right ventricular cardiomyopathy
K1 Genotype
K1 Risk stratification
K1 Sudden cardiac death
K1 Ventricular arrhythmia
AB 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: PKP2 (n = 118, 21%); desmoplakin (DSP) (n = 79, 14%); other desmosomal (n = 59, 11%); and gene elusive (n = 160, 29%). Discrimination and calibration were highest for PKP2 and lowest for the gene-elusive group. Univariable analyses revealed the variable performance of individual clinical risk markers in the different gene groups, e.g. right ventricular dimensions and systolic function are significant risk markers in PKP2 but not in DSP patients and the opposite is true for left ventricular systolic function. The 2019 ARVC risk model performs reasonably well in gene-positive ARVC (particularly for PKP2) but is more limited in gene-elusive patients. Genotype should be included in future risk models for ARVC.
YR 2022
FD 2022
LK http://hdl.handle.net/10668/19768
UL http://hdl.handle.net/10668/19768
LA en
DS RISalud
RD Apr 4, 2025