Details

Autor(en) / Beteiligte

• Protonotarios, Alexandros
• Bariani, Riccardo
• Cappelletto, Chiara
• Pavlou, Menelaos
• García-García, Alba
• Cipriani, Alberto
• Protonotarios, Ioannis
• Rivas, Adrian
• Wittenberg, Regitze
• Graziosi, Maddalena
• Xylouri, Zafeirenia
• Larrañaga-Moreira, José M
• de Luca, Antonio
• Celeghin, Rudy
• Pilichou, Kalliopi
• Bakalakos, Athanasios
• Lopes, Luis Rocha
• Savvatis, Konstantinos
• Stolfo, Davide
• Dal Ferro, Matteo
• Merlo, Marco
• Basso, Cristina
• Freire, Javier Limeres
• Rodriguez-Palomares, Jose F
• Kubo, Toru
• Ripoll-Vera, Tomas
• Barriales-Villa, Roberto
• Antoniades, Loizos
• Mogensen, Jens
• Garcia-Pavia, Pablo
• Wahbi, Karim
• Biagini, Elena
• Anastasakis, Aris
• Tsatsopoulou, Adalena
• Zorio, Esther
• Gimeno, Juan R
• Garcia-Pinilla, Jose Manuel
• Syrris, Petros
• Sinagra, Gianfranco
• Bauce, Barbara
• Elliott, Perry M

Titel

Importance of genotype for risk stratification in arrhythmogenic right ventricular cardiomyopathy using the 2019 ARVC risk calculator

Ist Teil von

• European heart journal, 2022-08, Vol.43 (32), p.3053-3067

Ort / Verlag

England: Oxford University Press

Erscheinungsjahr

2022

Link zum Volltext

Quelle

Oxford Journals 2020 Medicine

Beschreibungen/Notizen

• 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.