Key result
Novel risk score accurately predicts major arrhythmic events in non-dilated left ventricular cardiomyopathy with 0.81 C-index.
Why the study?
The prediction of the first major arrhythmic event in non-dilated left ventricular cardiomyopathy remains an unmet clinical need.
Does a novel risk score predict the first major arrhythmic event in patients with non-dilated left ventricular cardiomyopathy?
Cohort (n=553)
Yes
Does a novel risk score predict the first major arrhythmic event in patients with non-dilated left ventricular cardiomyopathy?
Effect estimate: C-index 0.81 (95% CI 0.75-0.88)
A novel multiparametric risk score accurately predicts the first major arrhythmic event in patients with non-dilated left ventricular cardiomyopathy, potentially guiding primary prevention ICD placement.
Identifies predictors of MAE in NDLVC; hypothesis-generating for a novel score, pending prospective validation before clinical use.
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.
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Peretto et al. (2025) conducted a cohort in Non-dilated left ventricular cardiomyopathy (NDLVC) (n=553). Novel risk prediction score (clinical, imaging, and genetic factors) vs. Lower risk scores / absence of risk factors was evaluated on First major arrhythmic event (sustained VT, VF, or appropriate ICD therapy) by 60 months (C-index 0.81, 95% CI 0.75-0.88). A novel risk score using clinical, imaging, and genetic factors predicted first major arrhythmic events in non-dilated left ventricular cardiomyopathy (validation C-index 0.81; 95% CI 0.75-0.88).
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