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 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.

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Journal
Journal of Cardiovascular Development and Disease
Published
2025-03-16
DOI
https://doi.org/10.3390/jcdd12030103
Primary Topic
Hemoglobinopathies and Related Disorders
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article
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article

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

Antonella Meloni, Anna Spasiano, Nicolò Schicchi, Antonino Vallone et al.
Journal of Cardiovascular Development and Disease
Hemoglobinopathies and Related Disorders
article

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

Antonella Meloni, Anna Spasiano, Nicolò Schicchi, Antonino Vallone, Michele Santodirocco, Gennaro Restaino, Emanuele Grassedonio, Michele Emdin, Alberto Clemente, Laura Pistoia, Andrea Barison, Zelia Borsellino, Francesco Sorrentino, Valerio Cecinati, Giuseppe Messina, Vincenzo Positano
article en

Abstract

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.

Journal of Cardiovascular Development and DiseaseVol. 12(3)
Scuola Superiore Sant'Anna (IT), Casa Sollievo della Sofferenza (IT), Azienda ospedaliera "Bianchi-Melacrino-Morelli" (IT), Ospedale Garibaldi (IT), Azienda Ospedaliero Universitaria Ospedali Riuniti (IT), Presidio Ospedaliero (IT), St. Eugenio Hospital (IT), Azienda di Rilievo Nazionale ed Alta Specializzazione (IT), Azienda Ospedaliera Universitaria Policlinico "Paolo Giaccone" di Palermo (IT), Ospedale SS. Annunziata (IT), Fondazione Toscana Gabriele Monasterio (IT), Ospedali Riuniti Umberto I (IT), Regione Toscana
Good health and well-being
Openalex Percentile: Top 92%
Hemoglobinopathies and Related Disorders
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