Gedikli Omer, Altinbas Ahmet, Orucoglu Aslihan, Dogan Abdullah, Ozaydin Mehmet, Aslan Suleyman Murat, Acar Gurkan, Canatan Duran
Department of Cardiology, Sevket Demirel Heart Center, Suleyman Demirel University, Isparta, Turkey.
Echocardiography. 2007 Sep;24(8):830-6. doi: 10.1111/j.1540-8175.2007.00486.x.
Beta-thalassemia major (beta-TM) is a congenital hemolytic disorder characterized by impaired left ventricular and endothelial function. However, elastic properties of the aorta have not been sufficiently investigated in patients with beta-TM. We investigated whether beta-TM is related to impaired ascending aortic elastic properties.
We studied 36 patients with beta-TM (age: 15.8 +/- 2.6 years) and 30 age- and sex-matched control subjects by echocardiography. Aortic elastic indexes, aortic strain (%), distensibility (cm(2) dyn(-1) 10(-3)), and stiffness index were calculated from the echocardiographically derived thoracic aortic diameters (mm/m(2)), and the measurement of pulse pressure obtained by cuff sphygmomanometry.
Patients versus control subjects had increased aortic diameters (P < 0.001), lower mean aortic strain (9 +/- 3.6 vs. 14.9 +/- 3.2, P < 0.001) and distensibility (0.6 +/- 0.36 vs. 0.8 +/- 0.2, P < 0.012), and higher mean stiffness index (5.3 +/- 2.4 vs. 2.8 +/- 0.6, P < 0.001). Aortic elastic indexes were significantly associated with ferritin level, while stiffness index was significantly related to platelet count.
Elastic properties of ascending aorta are impaired in patients with beta-TM. Impaired functions of aorta may lead to deterioration of left ventricular function.
重型β地中海贫血（β-TM）是一种先天性溶血性疾病，其特征为左心室和内皮功能受损。然而，β-TM患者的主动脉弹性特性尚未得到充分研究。我们调查了β-TM是否与升主动脉弹性特性受损有关。
我们通过超声心动图研究了36例β-TM患者（年龄：15.8±2.6岁）和30例年龄及性别匹配的对照受试者。根据超声心动图得出的胸主动脉直径（mm/m²）以及袖带血压计测得的脉压，计算主动脉弹性指数、主动脉应变（%）、扩张性（cm² dyn⁻¹ 10⁻³）和硬度指数。
与对照受试者相比，患者的主动脉直径增加（P < 0.001），平均主动脉应变较低（9±3.6对14.9±3.2，P < 0.001）和扩张性较低（0.6±0.36对0.8±0.2，P < 0.012），平均硬度指数较高（5.3±2.4对2.8±0.6，P < 0.001）。主动脉弹性指数与铁蛋白水平显著相关，而硬度指数与血小板计数显著相关。
β-TM患者的升主动脉弹性特性受损。主动脉功能受损可能导致左心室功能恶化。
