Instituto de Investigación Sanitaria La Fe (IIS La Fe)
Published January 1, 2026 | Version v1
Journal article Open

Major arrhythmias in non-dilated left ventricular cardiomyopathy: a novel prediction score

Authors/Creators

  • 1. Inherited Cardiac Diseases Unit, Cardiology Department, Hospital Universitario y Politécnico La Fe, Valencia, Spain

Description

Background and Aims The prediction of the first major arrhythmic event (MAE) is still an unmet need in the recently defined scenario of non-dilated left ventricular cardiomyopathy (NDLVC). Methods A cohort of 337 patients with NDLVC and no history of MAE was retrospectively identified at two large centres. Patient-tailored diagnostic workup included cardiac magnetic resonance (CMR), endomyocardial biopsy, and genetic testing. The primary endpoint was the occurrence of the first MAE, including sustained ventricular tachycardia (VT), ventricular fibrillation, or appropriate implantable cardioverter-defibrillator therapy, by 60-month follow-up. A pool of 216 NDLVC patients from 11 European centres served as a validation cohort. Results In the study cohort (mean age 37 +/- 15 years, 62% male), the mean left ventricular ejection fraction (LVEF) was 52 +/- 8%, and 79% of patients had late gadolinium enhancement (LGE) at baseline CMR. By 60-month follow-up, 51 patients (15%) experienced a MAE. The primary endpoint was predicted by male sex [hazard ratio (HR) 2.4, 95% confidence interval (CI) 1.3-4.4, P = .007], baseline non-sustained VT (HR 3.1, 95% CI 1.7-5.6, P < .001), LVEF < 45% (HR 5.5, 95% CI 2.7-11.0, P < .001), septal (HR 2.0, 95% CI 1.0-4.0, P = .046) and ring-like pattern of LGE (HR 1.3, 95% CI .6-2.6, P = .54), pathogenic/likely pathogenic variants in guideline-defined high-risk genes (HR 4.6, 95% CI 2.3-9.1, P < .001), and biopsy/CMR-proven myocardial inflammation (HR 15.7, 95% CI 6.1-40.3, P < .001). The results were confirmed in the validation cohort (Uno's C-index 0.81, 95% CI .75-.88). A novel risk score was subsequently derived. Conclusions In NDLVC, male sex, non-sustained VT, LVEF < 45%, septal and ring-like LGE, high-risk genotypes, and myocardial inflammation predicted the first episode of MAE by 60 months.

Notes

Funding text 1: This work was performed during G.P.\u2019s tenure as the Clinical Research Award in Honour of Mark Josephson and Hein Wellens Fellow of the Heart Rhythm Society. B.H. is a participant in the Berlin Institute of Health-Charit\u00E9 Advanced Clinician Scientist Pilot Program funded by the Charit\u00E9\u2013Universit\u00E4tsmedizin Berlin and the Berlin Institute of Health.; Funding text 2: G.P. received speaker fees from Abbott. M.Ca. received speaker fees from Abbott and Biosense Webster. B.H. is an inventor of patents that use RNA for the diagnosis of myocarditis (patent protection is being processed for MCG for diagnosis and measurement of therapy response in inflammatory cardiomyopathy and for cytokines as therapy targets in inflammatory cardiomyopathy). P.D.B. is a consultant for Abbott and Biosense and has received research grants from Abbott, Biosense, Biotronik, and Boston Scientific. The other authors report no disclosures.

Files

10.1093eurheartjehaf477.pdf

Files (1.5 MB)

Name Size Download all
md5:85ff5c727476b71ebc4a6fe90ad8abb0
1.5 MB Preview Download