Arrhythmic genotypes in dilated cardiomyopathy and risk of advanced heart failure

Background and aims Certain genetic forms of dilated cardiomyopathy (DCM) entail a higher arrhythmic risk. It is unknown whether DCM patients with high-risk arrhythmic genotypes also develop more advanced heart failure (AHF) complications. AHF events were studied according to DCM genotype.Methods Cl...

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Autores: Mora-Ayestarán, N, Ochoa, JP, Gómez-González, C, Navarro-Peñalver, M, Gallego-Delgado, M, Larrañaga-Moreira, JM, Robles-Mezcua, A, Basurte-Elorz, MT, Rodriguez-Palomares, JF, Climent-Paya, V, Jiménez-Jaímez, J, Mogollón-Jiménez, MV, García-Granja, PE, García-Alvarez, A, Peña-Peña, ML, Barredo, MA, Ripoll-Vera, T, Palomino-Doza, J, Bayes-Genis, A, Tirón, C, Fernández, AI, Sabater-Molina, M, Toranzo, I, Crespo-Leiro, MG, Doncel-Abad, V, Lacuey-Lecumberri, G, Limeres-Freire, J, García-Alvarez, M, Cabrera-Borrego, E, El Maalem, ZKA, Vilches, S, González-López, E, Villacorta, E, García-Pinilla, JM, Barriales-Villa, R, Gimeno-Blanes, JR, Garcia-Pavia, P, Domínguez, F
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2025
País:España
Institución:Instituto de Investigación Biomédica y Sanitaria de Alicante (ISABIAL)
Repositorio:r-ISABIAL. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica y Sanitaria de Alicante
OAI Identifier:oai:isabial.fundanetsuite.com:p11304
Acceso en línea:https://isabial.portalinvestigacion.com/publicaciones11304
https://doi.org/10.1093/eurheartj/ehaf605
Access Level:acceso abierto
Palabra clave:Dilated cardiomyopathy
Genes
Heart failure
Sudden cardiac death
Prognosis
Descripción
Sumario:Background and aims Certain genetic forms of dilated cardiomyopathy (DCM) entail a higher arrhythmic risk. It is unknown whether DCM patients with high-risk arrhythmic genotypes also develop more advanced heart failure (AHF) complications. AHF events were studied according to DCM genotype.Methods Clinical data from 1203 genotyped DCM patients were collected from 19 Spanish centres. Patients were classified into high-risk arrhythmic genotypes (LMNA, FLNC, desmosomal genes, PLN, TMEM43, RBM20), TTN, other genes, and genotype negative (Gen-). The primary endpoint was a composite of AHF events (ventricular assist device implantation, heart transplant, and AHF-related mortality). The secondary endpoint was a combination of malignant ventricular arrhythmias (MVA).Results A DCM-causing variant was identified in a high-risk arrhythmic gene in 185 patients (15.4%), 193 (16.0%) had variants in TTN, 134 (11.1%) in other genes, and 691 (57.4%) were Gen-. After a median follow-up of 5.7 years (interquartile range 2.9-9.1 years), AHF events occurred in 45 (24.3%) patients in the high-risk arrhythmic group, while in 25 (18.7%), 25 (13.0%), and 70 (10.1%) patients with other genotypes, TTN, and Gen-, respectively (hazard ratio 1.85, 95% confidence interval 1.31-2.61 for high-risk arrhythmic genes compared with other groups). MVA occurred in 55 patients (29.7%) (hazard ratio 2.52, 95% confidence interval 1.81-3.51 for high-risk genotypes vs other groups). High-risk arrhythmic genotype was the main independent predictor of AHF in multivariate analysis. High-risk arrhythmic genotype and late gadolinium enhancement were independent predictors of MVA.Conclusions Patients with high-risk arrhythmic genotypes also experience more AHF events, supporting a differential therapeutic approach in this group of patients beyond sudden death prevention.

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