| Autor: |
Pistoia L; Unità Operativa Complessa Ricerca Clinica, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy.; Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy., Meloni A; Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy.; Bioengineering Unit, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy., Positano V; Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy.; Bioengineering Unit, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy., Longo F; Unità Operativa Day Hospital della Talassemia e delle Emoglobinopatie, Azienda Ospedaliero-Universitaria 'S. Anna', 44124 Ferrara, Italy., Borsellino Z; Unità Operativa Complessa Ematologia con Talassemia, ARNAS Civico 'Benfratelli-Di Cristina', 90134 Palermo, Italy., Spasiano A; Unità Operativa Semplice Dipartimentale Malattie Rare del Globulo Rosso, Azienda Ospedaliera di Rilievo Nazionale 'A. Cardarelli', 80131 Napoli, Italy., Righi R; Diagnostica per Immagini e Radiologia Interventistica, Ospedale del Delta, 44023 Ferrara, Italy., Renne S; Struttura Complessa di Cardioradiologia-UTIC, Presidio Ospedaliero 'Giovanni Paolo II', 88046 Cosenza, Italy., Izzo D; Unità Operativa Complessa di Cardiologia-UTIC, Presidio Ospedaliero 'D.ssa Anastasia Guerriero', 81025 Caserta, Italy., Savino K; Sezione di Cardiologia e Fisiopatologia Cardiovascolare, Dipartimento di Medicina e Chirurgia, Università degli Studi di Perugia, 06132 Perugia, Italy., Mavrogeni S; Onassis Cardiac Surgery Center, 17674 Athens, Greece., Quaia E; Istituto di Radiologia, Dipartimento di Medicina, Università di Padova, 35128 Padova, Italy., Cademartiri F; Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy., Pepe A; Istituto di Radiologia, Dipartimento di Medicina, Università di Padova, 35128 Padova, Italy. |
| Abstrakt: |
Cardiac involvement in sickle beta thalassemia (Sβ-thal) patients has been poorly investigated. We aimed to evaluate cardiac function and myocardial iron overload by cardiovascular magnetic resonance (CMR) in patients with Sβ-thal. One-hundred and eleven Sβ-thal patients consecutively enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) network were studied and compared with 46 sickle cell anemia (SCA) patients and with 111 gender- and age- matched healthy volunteers. Cine images were acquired to quantify biventricular function. Myocardial iron overload (MIO) was assessed by the T2* technique, while macroscopic myocardial fibrosis was evaluated by the late gadolinium enhancement (LGE) technique. In Sβ-thal and SCA patients, the morphological and functional CMR parameters were not significantly different, except for the left atrial area and left ventricular (LV) stroke volume, indexed by body surface area ( p = 0.023 and p = 0.048, respectively), which were significantly higher in SCA patients. No significant differences between the two groups were found in terms of myocardial iron overload and macroscopic myocardial fibrosis. When compared to healthy subjects, Sβ-thal patients showed significantly higher bi-atrial and biventricular parameters, except for LV ejection fraction, which was significantly lower. The CMR analysis confirmed that Sβ-thal and SCA patients are phenotypically similar. Since Sβ-thal patients showed markedly different morphological and functional indices from healthy subjects, it would be useful to identify Sβ-thal/SCA-specific bi-atrial and biventricular reference values. |
