Sau F, Lai M E, Seguro C, Onnis E, Figus A, Farci P, Balestrieri A, Cherchi A
Cardiologia. 1989 Mar;34(3):221-7.
An echocardiographic study was performed in 21 young patients with thalassemia major (TM, age 16-22 years), with no cardiac symptoms, and in 24 age-matched normals (N) at rest and during sitting bicycle exercise (EX). All TM were receiving blood transfusions regularly to maintain hemoglobin level above 11 g/dl and subcutaneous infusion of desferrioxamine (40 mg/Kg/day) to reduce hemosiderosis. At rest, in comparison to N, TM showed a significant increase in LV end-diastolic dimension (EDD), septal, posterior wall thickness and mass, whereas wall thickness to EDD ratio and LV fractional shortening (FS) were not different. Stress echocardiography was recorded with success in 90% of TM and 83% of N. During EX, FS increased progressively with increase of heart rate in both groups, though the increase of FS was lower in TM. Thus, asymptomatic young patients with thalassemia major, under well transfusion-chelation therapy, showed cardiac changes from volume overload. LV systolic function, normal at rest, was moderately impaired during exercise test.
对21名无心脏症状的重型地中海贫血(TM,年龄16 - 22岁)年轻患者以及24名年龄匹配的正常对照者(N)在静息状态及进行坐位自行车运动(EX)期间进行了超声心动图研究。所有TM患者均定期接受输血以维持血红蛋白水平高于11 g/dl,并皮下输注去铁胺(40 mg/Kg/天)以减少铁过载。静息时,与N相比,TM患者的左心室舒张末期内径(EDD)、室间隔、后壁厚度及质量显著增加,而壁厚与EDD比值及左心室缩短分数(FS)无差异。90%的TM患者和83%的N成功记录到了负荷超声心动图。运动期间,两组的FS均随心率增加而逐渐升高,尽管TM患者FS的升高幅度较小。因此,接受良好输血 - 螯合治疗的无症状重型地中海贫血年轻患者表现出容量负荷过重导致的心脏改变。左心室收缩功能在静息时正常,但在运动试验期间中度受损。