Meloni Antonella, Pistoia Laura, Spasiano Anna, Sorrentino Francesco, Messina Giuseppe, Santodirocco Michele, Borsellino Zelia, Cecinati Valerio, Positano Vincenzo, Restaino Gennaro, Schicchi Nicolò, Grassedonio Emanuele, Vallone Antonino, Emdin Michele, Clemente Alberto, Barison Andrea
Bioengineering Unit, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy.
Unità Operativa Semplice Dipartimentale Ricerca Clinica, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy.
J Cardiovasc Dev Dis. 2025 Mar 16;12(3):103. doi: 10.3390/jcdd12030103.
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; = 294), DCM ( = 12), and NDLVC ( = 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.
我们研究了输血依赖型β地中海贫血(β-TDT)患者中扩张型心肌病(DCM)和非扩张型左心室心肌病(NDLVC)的患病率、临床特征及预后作用。我们回顾性纳入了415例接受心血管磁共振检查的β-TDT患者,以量化心肌铁过载(MIO)和双心室功能参数,并检测替代性心肌纤维化。在无明显心肌病(NOCM;n = 294)、DCM(n = 12)和NDLVC(n = 109)的患者中,人口统计学和实验室参数具有可比性,而心脏大小和收缩功能存在显著差异。与NOCM患者相比,DCM和NDLVC患者的MIO和替代性心肌纤维化患病率更高。在平均57.03±18.01个月的随访期间,32例(7.7%)患者出现心脏并发症:15例心力衰竭、15例室上性心律失常和2例肺动脉高压。与NOCM组相比,NDLVC组和DCM组发生心脏并发症的风险均显著增加(风险比分别为4.26和8.81)。在多变量分析中,独立预测因素为年龄、MIO以及DCM和NDLVC相对于NOCM表型的存在情况。在β-TDT中,检测NDLVC和DCM表型可能对预测心脏结局具有价值。