Key result
Multiparametric model incorporating genetics, clinical, and CMR features predicts NDLVC progression with an AUC of ~0.84.
Why the study?
Non-dilated left ventricular cardiomyopathy is a highly heterogeneous and largely unexplored category of cardiomyopathies introduced in the 2023 ESC guidelines.
A multiparametric model incorporating clinical, electrocardiographic, imaging, genetic, and histological parameters effectively stratifies the risk of disease progression in patients with non-dilated left ventricular cardiomyopathy.
Background Non-Dilated Left Ventricular Cardiomyopathy (NDLVC) is a highly heterogeneous and largely unexplored category of cardiomyopathies introduced in the 2023 European Society of Cardiology guidelines. Aim This study aimed to identify factors associated with adverse remodeling in a large multicenter cohort of patients with NDLVC. Methods 432 NDLVC patients (63.4% male, mean age 39.4 ± 14.6 years) with multiparametric characterization were enrolled from January 2010 to May 2023 in two high-volume Italian centers. Diagnosis followed ESC criteria. The study endpoints were (I) worsening of left ventricular ejection fraction (LVEF) ≥10% from the baseline value, (II) evolution to dilated cardiomyopathy (DCM), and (III) a composite of both, assessed at the latest available echocardiographic evaluation for each patient. Results During a median follow-up of 77 months (IQR: 48-109), 118 patients (27.3%) experienced worsening LVEF, 125 (28.9%) progressed to DCM, and 79 (18.3%) reached the composite endpoint. A multivariable model including positive genetic testing, mostly when associated to the detection of myocardial inflammation (M-Infl) at endomyocardial biopsy or cardiac magnetic resonance (CMR), family history of cardiomyopathies and/or sudden cardiac death, intraventricular conduction delay, baseline echocardiographic LVEF <45%, a ring-like late gadolinium enhancement on CMR, and non-sustained ventricular tachycardia at baseline, had the strongest discrimination power for predicting worsening LVEF [AUC of 0.8 (95% CI: 0.75-0.86)], evolution to DCM [AUC 0.78 (95% CI: 0.73-0.84)] and the composite of both [AUC 0.84 (95% CI: 0.79-0.89)]. The absence of genetic variants reduced the risk across all the endpoints [(OR: 0.2, 95% CI: 0.1-0.4, p<0.001), (OR: 0.2, 95% CI: 0.1-0.4, p<0.001) and (OR: 0.1, 95% CI: 0.04-0.3, p<0.001), respectively]. These findings were confirmed in the sensitivity analysis on patients with baseline echocardiographic LVEF ≥50%. Conclusion In this large NDLVC longitudinal cohort, a multiparametric model, including clinical, electrocardiographic, imaging, genetic and histological parameters, effectively stratifies the risk of disease progression.
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Bacigalupi et al. (2025) studied this question. A multiparametric model including genetics, myocardial inflammation, family history, ECG, LVEF, and CMR predicts NDLVC progression with AUCs up to 0.84 and ORs down to 0.1 for risk reduction.
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