"Comparison of cardiac status in Thalassemic patients with that of non-Thalassemic children "
Abstract
Cardiac siderosis from transfused iron remained, despite iron chelation therapy with desferioxamine (DFO), the major cause of death in Thalassemia major. In a retrospective study, we reviewed 67 patients with Thalassemia major and the same number of non-thalassemic children and analyzed factors affecting cardiac involvement. Ejection fraction (EF) and shortening fraction (SF) of Thalassemic group was lower than that of non-thalassemic and difference was significant (P<0.001). In Thalassemic patients there was inverse relationship between serum ferritin and cardiac OF and SF. Patients with optimal compliance had lower serum ferritin (P<0.001). 16 patients showed some kind of cardiac involvement. Older (P<0.001), had lower SF (P<0.05) and late onset of DFO therapy (P<0.001). We concluded that patients with Thalassemia major, despite iron chelation therapy with DFO, had weaker cardiac performance compared to non-thalassemic children. In these patients cardiac complications occurred in older age group with late onset of DFO treatment. Echocardiographic measurement of SF has prognostic value.
Keywords
Thalassemia major, Desferal, Desferioxamine, Cardiomyopathy,
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