b4dcbb7256e8f9e0a22aa3b6f32a4a3e ehaf605.pdf 20f565544a9b99099ffde3b2d05b137ad54bbd50 ehaf605.pdf 1ff683b4c06e7a7e3dca6c06e605f61721b5dfae17f76f0f3cd636df0e1135fd ehaf605.pdf Title: Arrhythmic genotypes in dilated cardiomyopathy and risk of advanced heart failure Subject: DOI: 10.1093/eurheartj/ehaf605; European Heart Journal, 46, 48, 2025-08-29.; Abstract: 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 Keywords: Dilated cardiomyopathy; Genes; Heart failure; Sudden cardiac death; Prognosis Author: Nerea Mora-Ayestarán Creator: Servigistics Arbortext Advanced Print Publisher 11.1.4546/W-x64 Producer: PDFlib+PDI 9.0.7p3 (C++/Win64) CreationDate: Sat Dec 13 11:48:52 2025 CET ModDate: Thu Dec 18 13:26:01 2025 CET Custom Metadata: yes Metadata Stream: yes Tagged: yes UserProperties: no Suspects: no Form: none JavaScript: no Pages: 12 Encrypted: no Page size: 595.276 x 793.701 pts Page rot: 0 File size: 833568 bytes Optimized: yes PDF version: 1.6 name type encoding emb sub uni object ID ------------------------------------ ----------------- ---------------- --- --- --- --------- RRLAME+GillSansNova-Book CID TrueType Identity-H yes yes yes 2168 0 DCODHU+GillSansNova-Bold CID TrueType Identity-H yes yes yes 2171 0 ZCUAWU+GillSansNova-BookItalic CID 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Larrañaga-Moreira Ainhoa Robles-Mezcua María Teresa Basurte-Elorz Jose Fernando Rodriguez-Palomares Vicente Climent-Paya Juan Jiménez-Jaímez Maria Victoria Mogollón-Jiménez Pablo Elpidio García-Granja Ana García-Álvarez María Luisa Peña-Peña María Alvarez Barredo Tomas Ripoll-Vera Julián Palomino-Doza Antoni Bayes-Genis Coloma Tirón Ana Isabel Fernández María Sabater-Molina Inés Toranzo María G. Crespo-Leiro Victoria Doncel-Abad Gemma Lacuey-Lecumberri Javier Limeres-Freire Maria I. García-Álvarez Eva Cabrera-Borrego Zineb Kounka-Ait El Maalem Silvia Vilches Esther González-López Eduardo Villacorta José M. García-Pinilla Roberto Barriales-Villa Juan Ramón Gimeno-Blanes Pablo Garcia-Pavia Fernando Domínguez DOI: 10.1093/eurheartj/ehaf605; European Heart Journal, 46, 48, 2025-08-29.; Abstract: 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 © The Author(s) 2025. Published by Oxford University Press on behalf of the European Society of Cardiology. Dilated cardiomyopathy Genes Heart failure Sudden cardiac death Prognosis Journal European Heart Journal © The Author(s) 2025. 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