Prevalence and Correlates of Dilated and Non-Dilated Left Ventricular Cardiomyopathy in Transfusion-Dependent Thalassemia: Data from a National, Multicenter, Observational Registry

Prevalence and Correlates of Dilated and Non-Dilated Left Ventricular Cardiomyopathy in Transfusion-Dependent Thalassemia: Data from a National, Multicenter, Observational Registry

We investigated the prevalence, clinical characteristics, and prognostic role of dilated cardiomyopathy (DCM) and non-dilated left ventricular cardiomyopathy (NDLVC) in patients with transfusion-dependent β-thalassemia (β-TDT). We retrospectively included 415 β-TDT patients who underwent cardiovascu...

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Main Authors: Antonella Meloni, Laura Pistoia, Anna Spasiano, Francesco Sorrentino, Giuseppe Messina, Michele Santodirocco, Zelia Borsellino, Valerio Cecinati, Vincenzo Positano, Gennaro Restaino, Nicolò Schicchi, Emanuele Grassedonio, Antonino Vallone, Michele Emdin, Alberto Clemente, Andrea Barison
Format: Article
Language:English
Published: MDPI AG 2025-03-01
Series:Journal of Cardiovascular Development and Disease
Subjects:
transfusion-dependent β-thalassemia
cardiovascular magnetic resonance
dilated cardiomyopathy
non-dilated left ventricular cardiomyopathy
prognosis
Online Access:https://www.mdpi.com/2308-3425/12/3/103
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Summary:We investigated the prevalence, clinical characteristics, and prognostic role of dilated cardiomyopathy (DCM) and non-dilated left ventricular cardiomyopathy (NDLVC) in patients with transfusion-dependent β-thalassemia (β-TDT). We retrospectively included 415 β-TDT patients who underwent cardiovascular magnetic resonance to quantify myocardial iron overload (MIO) and biventricular function parameters and to detect replacement myocardial fibrosis. Demographic and laboratory parameters were comparable among patients with no overt cardiomyopathy (NOCM; <i>n</i> = 294), DCM (<i>n</i> = 12), and NDLVC (<i>n</i> = 109), while cardiac size and systolic function were significantly different. Compared to NOCM patients, DCM and NDLVC patients had a higher prevalence of MIO and replacement myocardial fibrosis. During a mean follow-up of 57.03 ± 18.01 months, cardiac complications occurred in 32 (7.7%) patients: 15 heart failures, 15 supraventricular arrhythmias, and 2 pulmonary hypertensions. Compared to the NOCM group, both the NDLVC and the DCM groups were associated with a significantly increased risk of cardiac complications (hazard ratio = 4.26 and 8.81, respectively). In the multivariate analysis, the independent predictive factors were age, MIO, and the presence of DCM and NDLVC versus the NOCM phenotype. In β-TDT, the detection of NDLVC and DCM phenotypes may hold value in predicting cardiac outcomes.
ISSN:2308-3425

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