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. 2025 Jul 18:ehaf477.
doi: 10.1093/eurheartj/ehaf477. Online ahead of print.

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

Giovanni Peretto  1   2   3 , Marco Merlo  4 , Alessandro Ambrosi  3   5 , Elena Bacigalupi  4 , Andrea Villatore  1   3 , Lucia Molinari  1   3 , Ignasi Anguera  6   7 , Eduard Claver  6   7 , Matteo Dal Ferro  4 , Phillip Suwalski  8 , Michael Spartalis  9 , Job Verdonschot  10   11 , Michele Ciabatti  12 , Nicolò Martini  13 , Mattia Zampieri  14 , Alessia Paldino  4 , Yari Valeri  15 , Cinzia Radesich  4 , Davide Lazzeroni  16 , Filippo Maria Cauti  2 , Carlos Moliner-Abós  17 , Esther Zorio  18 , Raimondo Pittorru  13 , Massimo Slavich  19 , Giulia Bassetto  4 , Alberto Marchi  14 , Lina Manzi  4 , Chiara Di Resta  3   20 , Maria Perotto  4 , Carola Pio Loco  4 , Michela Casella  15   21 , Maurizio Pieroni  12   22 , Simone Sala  1   2 , Iacopo Olivotto  14 , Cristina Basso  23 , Martina Perazzolo Marra  13 , Antonio Esposito  1   3   24 , Bettina Heidecker  8   25 , Andrea Di Marco  6   7 , Stephane Heymans  10   26 , Paolo Della Bella  2 , Gianfranco Sinagra  4
Affiliations

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

Giovanni Peretto et al. Eur Heart J. .

Abstract

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.

Keywords: Cardiomyopathy; Inflammation; NDLVC; Risk stratification; Sudden cardiac death; Ventricular arrhythmia.

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