بررسی تاثیر مقادیر بالای دسفرال وریدی بر بهبود عوارض قلبی بیماران مبتلا به تالاسمی

Background and purpose: This study was undertaken to determine the effects of high dose IV desferal on symptoms, LV size and function in thalassemic patients Materials and methods : 15 thalassemic patients with cardiomyopathy all of whom were on cardiotonic drugs for at least one month, aged between 15-25 yr, with ferritin> 1200ng/ml and Hb>9gr/dl were included in the study. They recieved IV desferal of 130mg/kg/day (max 5gr) during 10-14 hr for 5 consequtive days. Cardiac evaluation including history, plysical exam, ECG and Echocardiography were performed just before recieving desferal, 2 days and one month after completing the regimen. In additon visual and auditory consultations were done before and after the intervention to detect probable side effects. Results : By administering high dose IV desferal, the cardiovascular symptoms reduced considerably and systolic function showed significant improvement so that a reduction in LVEF from 49.1 ± 1.8% to 58/8 ± 2.9% at first follow up and to 57.8 ± 2.1% at second follow up was observed. EPSS decreazed from 9.6 ± 0.8 mm to 6.7 ± 0.8 mm at first follow up and to 6.5 ± 0.6 mm at second follow up (p<0.001). However, there was no significant effect on diastolic function, ECG and physical findings. No remarkable visual and auditory side effects were demonstrated. Conclusion : Based on the data it can be concluded that high dose IV desferal has improving effects on thalassemic cardio myopathy with high ferritin level.