Background: Non-Dilated Left Ventricular Cardiomyopathy (NDLVC) is a highly heterogeneous and unexplored category of cardiomyopathies introduced in the 2023 European Society of Cardiology (ESC) guidelines. This study aims to identify factors associated with adverse remodeling in a large multicenter cohort of patients with NDLVC. Methods: 432 patients with NDLVC (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. Endpoints were (I) worsening of left ventricular ejection fraction (LVEF) ≥10% from the baseline value (or group W), (II) evolution to dilated cardiomyopathy (DCM) (or group E), and (III) the concomitant occurrence of both (or group W+E), assessed at the latest available echocardiographic evaluation. Results: During a median follow-up of 77 months (IQR:48-109), 27.3% of patients experienced worsening LVEF, 28.9% progressed to DCM, and 18.3% reached the combined W+E endpoint. A multivariable model including positive genetic testing associated to myocardial inflammation, family history of cardiomyopathies and/or sudden cardiac death, intraventricular conduction delay, baseline echocardiographic LVEF<45%, a ring-like late gadolinium enhancement on cardiac magnetic resonance, 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 combined W+E [AUC 0.84 (95% CI: 0.79-0.89)]. Negative genetic testing 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]. Conclusions: In this large NDLVC cohort, in which 38% of patients showed an adverse remodelling over long-term follow-up, a multiparametric approach integrating deep phenotyping and genetics effectively identified high-risk patients.
Predictors of Disease Progression in Patients with Left Ventricular Non-Dilated Cardiomyopathy / Bacigalupi, E., Merlo, M., Barbati, G., Villatore, A., Molinari, L., Dal Ferro, M., Paldino, A., Radesich, C., Bassetto, G., Manzi, L., Di Resta, C., Perotto, M., Pio Loco Detto Gava, C., Sala, S., Esposito, A., Della Bella, P., Sinagra, G., Peretto, G.. - In: CANADIAN JOURNAL OF CARDIOLOGY. - ISSN 0828-282X. - (2025), pp. "-"-"-". [Epub ahead of print] [10.1016/j.cjca.2025.12.013]
Predictors of Disease Progression in Patients with Left Ventricular Non-Dilated Cardiomyopathy
Merlo, Marco;Barbati, Giulia;Dal Ferro, Matteo;Paldino, Alessia;Perotto, Maria;Pio Loco Detto Gava, Carola;Sinagra, Gianfranco;
2025-01-01
Abstract
Background: Non-Dilated Left Ventricular Cardiomyopathy (NDLVC) is a highly heterogeneous and unexplored category of cardiomyopathies introduced in the 2023 European Society of Cardiology (ESC) guidelines. This study aims to identify factors associated with adverse remodeling in a large multicenter cohort of patients with NDLVC. Methods: 432 patients with NDLVC (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. Endpoints were (I) worsening of left ventricular ejection fraction (LVEF) ≥10% from the baseline value (or group W), (II) evolution to dilated cardiomyopathy (DCM) (or group E), and (III) the concomitant occurrence of both (or group W+E), assessed at the latest available echocardiographic evaluation. Results: During a median follow-up of 77 months (IQR:48-109), 27.3% of patients experienced worsening LVEF, 28.9% progressed to DCM, and 18.3% reached the combined W+E endpoint. A multivariable model including positive genetic testing associated to myocardial inflammation, family history of cardiomyopathies and/or sudden cardiac death, intraventricular conduction delay, baseline echocardiographic LVEF<45%, a ring-like late gadolinium enhancement on cardiac magnetic resonance, 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 combined W+E [AUC 0.84 (95% CI: 0.79-0.89)]. Negative genetic testing 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]. Conclusions: In this large NDLVC cohort, in which 38% of patients showed an adverse remodelling over long-term follow-up, a multiparametric approach integrating deep phenotyping and genetics effectively identified high-risk patients.Pubblicazioni consigliate
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