Predictors of Disease Progression in Patients with Left Ventricular Nondilated Cardiomyopathy
Elena Bacigalupi1, Marco Merlo2, Giulia Barbati3
1Cardiothoracovascular Department, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI) and University of Trieste, Trieste, Italy.
Insights
Nondilated left ventricular cardiomyopathy (NDLVC) is a new condition where adverse remodeling affects 38% of patients. Multiparametric analysis including genetics effectively identifies high-risk NDLVC patients for better management.
Area of Science:
- Cardiology
- Genetics
- Medical Diagnostics
Background:
- Nondilated left ventricular cardiomyopathy (NDLVC) is a newly defined, heterogeneous cardiomyopathy category per 2023 ESC guidelines.
- Limited data exists on factors driving adverse remodeling in NDLVC patients.
Purpose of the Study:
- To identify predictors of adverse cardiac remodeling in a large, multicenter NDLVC cohort.
- To evaluate the efficacy of a multiparametric approach in risk stratification.
Main Methods:
- 432 NDLVC patients underwent multiparametric characterization from 2010-2023.
- Endpoints included worsening left ventricular ejection fraction (LVEF), progression to dilated cardiomyopathy (DCM), or both.
- Multivariable analysis integrated clinical, echocardiographic, cardiac MRI, and genetic data.
Main Results:
- 38% of patients experienced adverse remodeling during a median 77-month follow-up.
- Positive genetic testing, family history, conduction delays, low baseline LVEF, specific MRI findings, and ventricular tachycardia predicted adverse outcomes.
- Negative genetic testing significantly reduced risk across all endpoints (OR 0.1-0.2).
Conclusions:
- A significant proportion of NDLVC patients experience adverse remodeling over time.
- Integrating deep phenotyping and genetic data is crucial for identifying high-risk NDLVC patients.
- This approach enables targeted interventions and improved patient management.
Background:
Nondilated 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 multicentre cohort of patients with NDLVC.
Methods:
A total of 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 2 high-volume Italian centres. Diagnosis followed ESC criteria. Endpoints were worsening of left ventricular ejection fraction (LVEF) ≥ 10% from the baseline value (or group W), evolution to dilated cardiomyopathy (DCM) (or group E), and 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 (interquartile range [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 or sudden cardiac death, intraventricular conduction delay, baseline echocardiographic LVEF < 45%, a ring-like late gadolinium enhancement on cardiac magnetic resonance, and nonsustained ventricular tachycardia at baseline had the strongest discrimination power for predicting worsening LVEF (area under the curve [AUC] of 0.8; 95% confidence interval [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 (odds ratio [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.
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