Meloni Antonella, Saba Luca, Cademartiri Filippo, Positano Vincenzo, Pistoia Laura, Cau Riccardo
Bioengineering Unit, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.
Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.
Radiol Med. 2024 Dec;129(12):1812-1822. doi: 10.1007/s11547-024-01916-6. Epub 2024 Nov 7.
Β-thalassemia major (TM) patients underwent regular transfusions to prevent complications of chronic anemia. However, these regular transfusions result in progressive iron accumulation in vital organs, including the heart. Myocardial iron overload can lead to cardiac dysfunction and ultimately to heart failure. Diagnosis of cardiac dysfunction in β-TM patients is usually made through clinical examination, electrocardiogram, and echocardiography. Cardiac magnetic resonance (CMR), through the measurement of T2* relaxation time, represents the diagnostic modality of choice for assessing myocardial iron overload and guiding the iron chelation therapy. Despite a tailored chelation therapy reducing myocardial iron overload, heart failure remains the leading cause of morbidity and mortality even in well-treated β-TM patients. Advances in CMR, including myocardial strain, parametric mapping (T1, T2, and extracellular volume), and late gadolinium enhancement (LGE) measurements, have expanded its role in the diagnosis, prognosis, and follow-up of these patients. This review seeks to offer a thorough overview of the potential uses of CMR in β-TM, extending beyond the established role of T2* measurement in guiding chelation therapy. It delves into the emerging applications of new CMR imaging biomarkers that could improve the overall management of β-TM patients.
重型β地中海贫血(TM)患者接受定期输血以预防慢性贫血的并发症。然而,这些定期输血会导致重要器官,包括心脏,铁逐渐蓄积。心肌铁过载可导致心脏功能障碍并最终发展为心力衰竭。β-TM患者心脏功能障碍的诊断通常通过临床检查、心电图和超声心动图进行。心脏磁共振成像(CMR)通过测量T2弛豫时间,是评估心肌铁过载和指导铁螯合治疗的首选诊断方法。尽管采用了量身定制的螯合疗法来减少心肌铁过载,但即使在治疗良好的β-TM患者中,心力衰竭仍然是发病和死亡的主要原因。CMR的进展,包括心肌应变、参数成像(T1、T2和细胞外容积)以及钆延迟增强(LGE)测量,扩大了其在这些患者诊断、预后和随访中的作用。本综述旨在全面概述CMR在β-TM中的潜在用途,超越T2测量在指导螯合治疗方面已确立的作用。它深入探讨了新的CMR成像生物标志物的新兴应用,这些应用可能改善β-TM患者的整体管理。