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.
Authors
- Antonella Meloni (ORCID: https://orcid.org/0000-0002-2284-8585)
- Anna Spasiano
- Nicolò Schicchi (ORCID: https://orcid.org/0000-0002-9851-9526)
- Antonino Vallone
- Michele Santodirocco (ORCID: https://orcid.org/0000-0002-0032-5628)
- Gennaro Restaino (ORCID: https://orcid.org/0000-0002-0392-4028)
- Emanuele Grassedonio (ORCID: https://orcid.org/0000-0002-2113-7300)
- Michele Emdin (ORCID: https://orcid.org/0000-0002-8541-1962)
- Alberto Clemente (ORCID: https://orcid.org/0000-0002-4285-9468)
- Laura Pistoia (ORCID: https://orcid.org/0000-0002-5319-2769)
- Andrea Barison (ORCID: https://orcid.org/0000-0001-9042-340X)
- Zelia Borsellino
- Francesco Sorrentino (ORCID: https://orcid.org/0000-0002-3460-3083)
- Valerio Cecinati (ORCID: https://orcid.org/0000-0001-8542-5240)
- Giuseppe Messina (ORCID: https://orcid.org/0000-0003-1060-1486)
- Vincenzo Positano (ORCID: https://orcid.org/0000-0001-6955-9572)
Institutions
- 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
Publication Details
- Journal
- Journal of Cardiovascular Development and Disease
- Published
- 2025-03-16
- DOI
- https://doi.org/10.3390/jcdd12030103
- Primary Topic
- Hemoglobinopathies and Related Disorders
- Type
- article
- Field-Weighted Citation Impact
- 0.00