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...
Saved in:
| Main Authors: | , , , , , , , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2025-03-01
|
| Series: | Journal of Cardiovascular Development and Disease |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2308-3425/12/3/103 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1849342159665233920 |
|---|---|
| author | 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 |
| author_facet | 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 |
| author_sort | Antonella Meloni |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-cbecd7eccb6c4e909326bbc7d7c10fa0 |
| institution | Kabale University |
| issn | 2308-3425 |
| language | English |
| publishDate | 2025-03-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Cardiovascular Development and Disease |
| spelling | doaj-art-cbecd7eccb6c4e909326bbc7d7c10fa02025-08-20T03:43:27ZengMDPI AGJournal of Cardiovascular Development and Disease2308-34252025-03-0112310310.3390/jcdd12030103Prevalence and Correlates of Dilated and Non-Dilated Left Ventricular Cardiomyopathy in Transfusion-Dependent Thalassemia: Data from a National, Multicenter, Observational RegistryAntonella Meloni0Laura Pistoia1Anna Spasiano2Francesco Sorrentino3Giuseppe Messina4Michele Santodirocco5Zelia Borsellino6Valerio Cecinati7Vincenzo Positano8Gennaro Restaino9Nicolò Schicchi10Emanuele Grassedonio11Antonino Vallone12Michele Emdin13Alberto Clemente14Andrea Barison15Bioengineering Unit, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, ItalyUnità Operativa Semplice Dipartimentale Ricerca Clinica, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, ItalyUnità Operativa Semplice Dipartimentale Malattie Rare del Globulo Rosso, Azienda Ospedaliera di Rilievo Nazionale “A. Cardarelli”, 80131 Napoli, ItalyUnità Operativa Semplice Dipartimentale Day Hospital Talassemici, Ospedale “Sant’Eugenio”, 00143 Roma, ItalyCentro Microcitemie, Grande Ospedale Metropolitano “Bianchi-Melacrino-Morelli”, 89100 Reggio Calabria, ItalyCentro Microcitemia—Day Hospital Thalassemia Poliambulatorio “Giovanni Paolo II”, Istituto di Ricovero e Cura a Carattere Scientifico Ospedale Casa Sollievo della Sofferenza, 71013 San Giovanni Rotondo, ItalyUnità Operativa Complessa Ematologia con Talassemia, Azienda di Rilievo Nazionale ad Alta Specializzazione Civico “Benfratelli-Di Cristina”, 90134 Palermo, ItalyStruttura Semplice di Microcitemia, Ospedale “SS. Annunziata”, 74123 Taranto, ItalyBioengineering Unit, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, ItalyRadiology Department, Responsible Research Hospital, 86100 Campobasso, ItalyDipartimento di Radiologia, Azienda Ospedaliero-Universitaria Ospedali Riuniti “Umberto I-Lancisi-Salesi”, 60020 Ancona, ItalySezione di Scienze Radiologiche—Dipartimento di Biopatologia e Biotecnologie Mediche, Policlinico “Paolo Giaccone”, 90127 Palermo, ItalyReparto di Radiologia, Azienda Ospedaliera “Garibaldi” Presidio Ospedaliero Nesima, 95126 Catania, ItalyCardiology and Cardiovascular Medicine, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, ItalyDepartment of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, ItalyCardiology and Cardiovascular Medicine, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, ItalyWe 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.https://www.mdpi.com/2308-3425/12/3/103transfusion-dependent β-thalassemiacardiovascular magnetic resonancedilated cardiomyopathynon-dilated left ventricular cardiomyopathyprognosis |
| spellingShingle | 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 Prevalence and Correlates of Dilated and Non-Dilated Left Ventricular Cardiomyopathy in Transfusion-Dependent Thalassemia: Data from a National, Multicenter, Observational Registry Journal of Cardiovascular Development and Disease transfusion-dependent β-thalassemia cardiovascular magnetic resonance dilated cardiomyopathy non-dilated left ventricular cardiomyopathy prognosis |
| title | Prevalence and Correlates of Dilated and Non-Dilated Left Ventricular Cardiomyopathy in Transfusion-Dependent Thalassemia: Data from a National, Multicenter, Observational Registry |
| title_full | Prevalence and Correlates of Dilated and Non-Dilated Left Ventricular Cardiomyopathy in Transfusion-Dependent Thalassemia: Data from a National, Multicenter, Observational Registry |
| title_fullStr | Prevalence and Correlates of Dilated and Non-Dilated Left Ventricular Cardiomyopathy in Transfusion-Dependent Thalassemia: Data from a National, Multicenter, Observational Registry |
| title_full_unstemmed | Prevalence and Correlates of Dilated and Non-Dilated Left Ventricular Cardiomyopathy in Transfusion-Dependent Thalassemia: Data from a National, Multicenter, Observational Registry |
| title_short | Prevalence and Correlates of Dilated and Non-Dilated Left Ventricular Cardiomyopathy in Transfusion-Dependent Thalassemia: Data from a National, Multicenter, Observational Registry |
| title_sort | prevalence and correlates of dilated and non dilated left ventricular cardiomyopathy in transfusion dependent thalassemia data from a national multicenter observational registry |
| topic | transfusion-dependent β-thalassemia cardiovascular magnetic resonance dilated cardiomyopathy non-dilated left ventricular cardiomyopathy prognosis |
| url | https://www.mdpi.com/2308-3425/12/3/103 |
| work_keys_str_mv | AT antonellameloni prevalenceandcorrelatesofdilatedandnondilatedleftventricularcardiomyopathyintransfusiondependentthalassemiadatafromanationalmulticenterobservationalregistry AT laurapistoia prevalenceandcorrelatesofdilatedandnondilatedleftventricularcardiomyopathyintransfusiondependentthalassemiadatafromanationalmulticenterobservationalregistry AT annaspasiano prevalenceandcorrelatesofdilatedandnondilatedleftventricularcardiomyopathyintransfusiondependentthalassemiadatafromanationalmulticenterobservationalregistry AT francescosorrentino prevalenceandcorrelatesofdilatedandnondilatedleftventricularcardiomyopathyintransfusiondependentthalassemiadatafromanationalmulticenterobservationalregistry AT giuseppemessina prevalenceandcorrelatesofdilatedandnondilatedleftventricularcardiomyopathyintransfusiondependentthalassemiadatafromanationalmulticenterobservationalregistry AT michelesantodirocco prevalenceandcorrelatesofdilatedandnondilatedleftventricularcardiomyopathyintransfusiondependentthalassemiadatafromanationalmulticenterobservationalregistry AT zeliaborsellino prevalenceandcorrelatesofdilatedandnondilatedleftventricularcardiomyopathyintransfusiondependentthalassemiadatafromanationalmulticenterobservationalregistry AT valeriocecinati prevalenceandcorrelatesofdilatedandnondilatedleftventricularcardiomyopathyintransfusiondependentthalassemiadatafromanationalmulticenterobservationalregistry AT vincenzopositano prevalenceandcorrelatesofdilatedandnondilatedleftventricularcardiomyopathyintransfusiondependentthalassemiadatafromanationalmulticenterobservationalregistry AT gennarorestaino prevalenceandcorrelatesofdilatedandnondilatedleftventricularcardiomyopathyintransfusiondependentthalassemiadatafromanationalmulticenterobservationalregistry AT nicoloschicchi prevalenceandcorrelatesofdilatedandnondilatedleftventricularcardiomyopathyintransfusiondependentthalassemiadatafromanationalmulticenterobservationalregistry AT emanuelegrassedonio prevalenceandcorrelatesofdilatedandnondilatedleftventricularcardiomyopathyintransfusiondependentthalassemiadatafromanationalmulticenterobservationalregistry AT antoninovallone prevalenceandcorrelatesofdilatedandnondilatedleftventricularcardiomyopathyintransfusiondependentthalassemiadatafromanationalmulticenterobservationalregistry AT micheleemdin prevalenceandcorrelatesofdilatedandnondilatedleftventricularcardiomyopathyintransfusiondependentthalassemiadatafromanationalmulticenterobservationalregistry AT albertoclemente prevalenceandcorrelatesofdilatedandnondilatedleftventricularcardiomyopathyintransfusiondependentthalassemiadatafromanationalmulticenterobservationalregistry AT andreabarison prevalenceandcorrelatesofdilatedandnondilatedleftventricularcardiomyopathyintransfusiondependentthalassemiadatafromanationalmulticenterobservationalregistry |