Meloni Antonella, Pistoia Laura, Longo Filomena, Spasiano Anna, Cecinati Valerio, Corigliano Elisabetta, Borsellino Zelia, Fotzi Ilaria, Positano Vincenzo, Zerbini Michela, Renne Stefania, Barbuto Luigi, Clemente Alberto, Ricchi Paolo
Bioengineering Unit, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy.
Unità Operativa Complessa Ricerca Clinica, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy.
J Clin Med. 2025 Jun 6;14(12):4020. doi: 10.3390/jcm14124020.
: We compared changes in hepatic and cardiac iron levels, left ventricular (LV) and right ventricular (RV) dimensions and function, and bi-atrial areas, all assessed through magnetic resonance imaging (MRI), between patients with non-transfusion-dependent thalassemia (NTDT) and those with neo-transfusion-dependent thalassemia (neo-TDT) over an 18-month follow-up period. : We included 32 NTDT patients (42.78 ± 12.62 years, 53.1% females) and 58 neo-TDT (>4 transfusions per year) patients (44.08 ± 14.13 years, 46.6% females), consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia project. Iron overload was quantified by T2* technique, biventricular function and atrial areas by cine images. Macroscopic myocardial fibrosis was detected by the late gadolinium enhancement technique. : Changes in cardiac and hepatic iron levels, in biventricular ejection fractions, in LV mass index, and bi-atrial areas were comparable between the two groups. A trend of worsening biventricular dimensions was observed in the NTDT group, while the neo-TDT group showed an improvement (decrease) in biventricular size (LV stroke volume index: = 0.036; LV cardiac index: = 0.031; RV end-diastolic volume index: = 0.034; RV stroke volume index: = 0.033). The inter-group comparison showed significant differences in the changes of biventricular end-diastolic volume indexes (LV: = 0.011 and RV: = 0.034) and stroke volume indexes (LV: = 0.036 and RV: = 0.033) and in the cardiac index ( < 0.0001). At both MRI scans, the frequency of replacement myocardial fibrosis was comparable between the two groups. : Our 18-month longitudinal data revealed distinct patterns of cardiac remodeling in NTDT and neo-TDT patients. The progressive ventricular dilation observed in NTDT patients highlights the need for careful MRI monitoring and potential interventions to address the long-term cardiac consequences of anemia.
我们比较了非输血依赖型地中海贫血（NTDT）患者和新输血依赖型地中海贫血（neo-TDT）患者在18个月随访期内肝脏和心脏铁水平的变化、左心室（LV）和右心室（RV）尺寸及功能，以及双心房面积，所有这些均通过磁共振成像（MRI）进行评估。我们纳入了32例NTDT患者（年龄42.78±12.62岁，女性占53.1%）和58例neo-TDT患者（每年输血>4次）（年龄44.08±14.13岁，女性占46.6%），这些患者均连续入选地中海贫血心肌铁过载扩展项目。铁过载通过T2*技术进行量化，双心室功能和心房面积通过电影图像进行评估。宏观心肌纤维化通过延迟钆增强技术进行检测。两组之间心脏和肝脏铁水平、双心室射血分数、左心室质量指数和双心房面积的变化具有可比性。在NTDT组中观察到双心室尺寸有恶化趋势，而neo-TDT组双心室大小有所改善（减小）（左心室每搏输出量指数：=0.036；左心室心脏指数：=0.031；右心室舒张末期容积指数：=0.034；右心室每搏输出量指数：=0.033）。组间比较显示双心室舒张末期容积指数（左心室：=0.011，右心室：=0.034）、每搏输出量指数（左心室：=0.036，右心室：=0.033）和心脏指数（<0.0001）的变化存在显著差异。在两次MRI扫描时，两组之间替代性心肌纤维化的频率具有可比性。我们的18个月纵向数据揭示了NTDT和neo-TDT患者心脏重塑的不同模式。NTDT患者中观察到的进行性心室扩张凸显了通过MRI进行仔细监测以及采取潜在干预措施以应对贫血长期心脏后果的必要性。
