Lewis B S, Lewis N, Dagan I, Rachmilewitz E A, Gotsman M S, Sapoznikov D
Isr J Med Sci. 1982 Sep;18(9):928-34.
Left-ventricular (LV) function was studied in 23 patients with anemia due to beta-thalassemia, of whom seven had thalassemia intermedia and the remainder thalassemia major. Two-thirds of the patients wih thalassemia intermedia and almost all the patients with thalassemia major were in clinical congestive heart failure. Despite this, resting measurements of ventricular size and systolic ventricular function were normal, indicating high-output cardiac failure. However, effort testing showed a flat response or decrease in the LV shortening fraction in patients with thalassemia major, and serial studies showed a decrease in the shortening fraction over a 4-yr period in some patients. LV diastolic function was studied by calculating peak LV filling rate and the pattern of LV filling in early diastole. Three patient with thalassemia major showed a pattern indicating abnormal LV distension. Since LV end-diastolic dimension was increased, volume overload was present in all patients. The results indicate that the following factors contribute to the genesis of cardiac failure in beta-thalassemia: 1) diminished response of systolic ventricular performance to exercise and later at rest; 2) ventricular volume overload; and 3) abnormal ventricular distension in diastole. Although the ventricular filling suggests abnormal LV compliance, the effect of right-ventricular volume overload or a pericardial factor cannot be excluded.
对23例β地中海贫血所致贫血患者的左心室（LV）功能进行了研究，其中7例为中间型地中海贫血，其余为重型地中海贫血。三分之二的中间型地中海贫血患者和几乎所有的重型地中海贫血患者都处于临床充血性心力衰竭状态。尽管如此，静息时心室大小和心室收缩功能的测量结果正常，提示高输出量心力衰竭。然而，运动试验显示重型地中海贫血患者的左心室缩短分数呈平坦反应或下降，系列研究显示部分患者在4年期间缩短分数下降。通过计算左心室峰值充盈率和舒张早期左心室充盈模式来研究左心室舒张功能。3例重型地中海贫血患者显示出提示左心室扩张异常的模式。由于左心室舒张末期内径增加，所有患者均存在容量超负荷。结果表明，以下因素促成了β地中海贫血中心力衰竭的发生：1）心室收缩功能对运动及后期静息状态的反应减弱；2）心室容量超负荷；3）舒张期心室扩张异常。虽然心室充盈提示左心室顺应性异常，但右心室容量超负荷或心包因素的影响不能排除。
