Echocardiographic studies in 44 subjects with thalassemia major(Thal) aged 5-27 years with body surface area(BSA) 0.8-1.9M2 were compared to 54 normals(N) of similar age and BSA. Hemoglobin of the Thals was maintained by transfusion at 11.4 gms.% ± 3.2 S.D. Of 39 Thals(Group 1) studied prior to Desferrioxamine therapy only 1 was on therapy for heart failure. Five patients (Group 11) aged 9-15 years were studied to assess left ventricular(LV) function because of recent onset of failure. In comparison to normal data 95% confidence limits, Group 1 subjects had increased LV end diastolic dimension(LVD) in 23/39(59%) and increased left atrial dimension(LAD) in 21/39(54%); LV wall thickness was above the mean normal values in 33/39(85%) patients. In Group 11, normal data 95% confidence limits were exceeded in 4/5 for LVD and 5/5 for LAD. Remarkably good LV function was found in Group 1 subjects, including 9 over 20 and 22 over 15 years of age. However, once heart failure was apparent(Group 11), LV function was markedly reduced and despite treatment for failure progression to death occurred within 1-11 months.
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Ehlers, K., Levin, A., Klein, A. et al. 100 EVALUATION OF PATIENTS WITH THALASSEMIA BY ECHOCARDIOGRAPHY. Pediatr Res 12, 380 (1978) doi:10.1203/00006450-197804001-00105