Multiparametric Cardiac Magnetic Resonance Assessment in Sickle Beta Thalassemia

Autor: Laura Pistoia, Antonella Meloni, Vincenzo Positano, Filomena Longo, Zelia Borsellino, Anna Spasiano, Riccardo Righi, Stefania Renne, Daniela Izzo, Ketty Savino, Sophie Mavrogeni, Emilio Quaia, Filippo Cademartiri, Alessia Pepe
Jazyk: angličtina
Rok vydání: 2024
Předmět:
Zdroj: Diagnostics, Vol 14, Iss 7, p 691 (2024)
Druh dokumentu: article
ISSN: 2075-4418
DOI: 10.3390/diagnostics14070691
Popis: 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.
Databáze: Directory of Open Access Journals
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