199 CARDIAC STATUS OF CHILDREN WITH SICKLE CELL ANEMIA (SS)

Abstract

Echocardiograms (E) were obtained from 44 children with SS and from 54 normal Blacks (Nl). Equations for predicting the normal values (PN) for 12 echo parameters were derived (PN = A . BSA^b . HR^c ± K), and the observed values from SS then expressed as standard deviation from normal (O PN). Increased cardiac output, LVEDD, LVESD, wall thickness, LA_max and Ao diameter were observed in SS (p < 0.001); however, the functional parameters %MAS, VCF and PEP/ET did not differ from Nl. Density function curves (DFC), derived by discriminant regression analysis using 7 echo parameters were obtained with equation: Y_dv = K₁ OPN₁ + K₂ OPN₂ + Kn OPN_n ± X, where Y_dv = discriminating value and K, X = constants. There was clear separation of the DFC of SS and Nl. Eleven children with SS underwent hypertransfusion therapy (HT) for 3/12-3 yrs (packed RBC q 2-3 weeks) to achieve Hgb ≥ 10 gm %, % S Hgb < 20%. E were obtained periodically before/after, and during HT. Mean values during HT/off HT were: Hgb 9.7/7.2 (p < .001), % S Hgb 12.1/88.2 (p < .001). The DFC and Y_dv before and after HT did not differ from those of 44 untreated SS (P = NS); during HT, there was significant shift (p < 0.001) of DFC and Y_dv towards Nl. We conclude that cardiac size and function are abnormal in SS children, improve during HT, but revert back to abnormal state after discontinuing the HT.

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