Combined Therapy with Desferal and Deferioprone in Improvement of Heart Function in Thalassemic Patients

Cardiac complications due to iron overload are the most common causes of death in patients with major thalassemia. This study assessed the efficacy of iron chelating by desferal-L1 in improvement of cardiac function in patients with major thalassemia. Methods and Materials : Patients older then 8 years old with major thalassemia that were admitted to hematology ward of Ali-e-Asghar hospital of Zahedan in 2005-2006 and had lower than normal diastolic indices in annual echocardiography were considered as study population. During primary tests, indices of diastolic function of left and right hearts were calculated, using echocardiography with 2D, M-mode and Doppler then the patients were placed on a combination of desferal (30-40mg/kg/day two nights per week) and L1 (deferiprone) (75mg/kg/day, three times a day). At the end of the study, cardiac indices were calculated again. Data were using SPSS software and paired t-test and p <0.05 was considered as significant level.

Of 38 patients, who were treated with a combination of desferal-L1, 19 were female with the mean age of 16.9 ± 3.1 and 18 were male with the mean age of 17.3 ± 2.6 years. Diastolic indices of right ventricle had no significant difference before and after treatment except PET/ET which decreased significantly after treatment (p <0.05). Of diastolic indices of left ventricule peak-E-velocity and IVRT and E/A ratio decreased significantly after treatment (p <0.05). t he difference of peak-A-velocity before and after treatment was not significant (p >0.05). Systolic indices of left ventricule increased significantly after treatment (p <0.05).

In this study, after one year of treatment with a combination of desferal-L1 in patients with major thalassemia, echocardiography showed improvement in left heart systolic and diastolic function. This combination therapy prevented progression of right ventricular diastolic function abnormality.