Abstract
Serial echocardiography was used to assess cardiac size and function of 5 children (0. 9 to 16 years) on hypertransfusion therapy (HT) for clinical problems related to SS. Satisfactory HT status, defined as total Hb levels maintained at ≥10 gm% and Hb-S at <10%, was achieved with washed packed RBC transfusion every 2-3 weeks. Iron status was monitored. Echo measurements of LV dimension (LVD), LA dimension (LAD), LV wall thickness (W), LV mass (M), cardiac output (Q), PEP/ET ratio and circumferential fiber shortening velocity (VCF) were obtained every 2-4 months during HT. These were compared to those of 100 normal children (48 Blacks, 52 Whites). Clinical improvement was dramatic. In one child (age 1 yr), the echo data remained normal before and during HT. The values in the other 4, expressed as % of predicted normal, before (C) and during HT for 8-10 mos are as follows:
The improved clinical status during HT is accompanied by diminution of cardiac size and of the high-output state. These changes may reverse after HT is discontinued.
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Lester, L., Moohr, J., Wilson, H. et al. 132 CARDIAC EFFECTS OF HYPERTRANSFUSION THERAPY IN SICKLE CELL DISEASE (SS). Pediatr Res 12 (Suppl 4), 385 (1978). https://doi.org/10.1203/00006450-197804001-00137
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DOI: https://doi.org/10.1203/00006450-197804001-00137