Chotivittayatarakorn P, Seksarn P, Pathmanand C, Thisyakorn C, Sueblinvong V
Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.
J Med Assoc Thai. 1993 Nov;76(11):591-6.
The biventricular systolic function was evaluated by M mode and Doppler echocardiography in 16 patients with homozygous beta-thalassemia and beta-thalassemia Hb E disease, aged 5-14 yrs (9.9 +/- 2.4 yr). The left ventricular end-diastolic dimension and left ventricular mass were increased in 88 per cent of the patients. Left ventricular fractional shortening and cardiac index were normal in all but one patient. Fifteen patients had evidence of pulmonary artery hypertension manifested by abnormality in the ratio of right ventricular acceleration time to ejection time (AT/ET). There was good correlation between platelets count and AT/ET (r = -0.70, P = 0.002). Thrombocytosis was noted in 4 patients. Our findings indicated that in beta-thalassemic children, right ventricular dysfunction was detected earlier than left ventricular dysfunction and platelets may play a role in the pathogenesis of pulmonary artery hypertension.
采用M型和多普勒超声心动图对16例5至14岁（平均9.9±2.4岁）的纯合子β地中海贫血和β地中海贫血Hb E病患者的双心室收缩功能进行了评估。88%的患者左心室舒张末期内径和左心室质量增加。除1例患者外，所有患者的左心室缩短分数和心脏指数均正常。15例患者有肺动脉高压的证据，表现为右心室加速时间与射血时间之比（AT/ET）异常。血小板计数与AT/ET之间存在良好的相关性（r = -0.70，P = 0.002）。4例患者出现血小板增多症。我们的研究结果表明，在β地中海贫血儿童中，右心室功能障碍比左心室功能障碍更早被检测到，并且血小板可能在肺动脉高压的发病机制中起作用。
