Abstract

14 patients (pts.), 3 to 27 years of age, were studied with M-mode echocardiography. 6 years ago transfusions (tx) were increased to maintain the pre-tx Hgb level above 10.5 g% with total iron loads (TIL) via tx of 35 to 508 g. The pts. were divided into Group A (7 pts. with TIL < 120 g) and Group B (7 pts. with TIL > 120 g). Two pts. had arrhythmias, and 3 had congestive failure with 2 deaths. The remainder had no cardiac symptoms. Positive echo findings are summarized below:Mean Vcf was 1.35±.14(SD) in Group A and 1.10±.26 in Group B (p<.025). 2 pts are alive 8 and 24 months after discovery of marked LV dysfunction, 1 pt. with normal LV function deteriorated rapidly and died 6 months later. In conclusion, RV and LA dilatation and LV hypertrophy occur commonly in Thalassemia pts. LV dilatation and dysfunction occur usually but unpredictably in pts. with TIL over 120 g. LV function indices do not seem predictive of survival time.

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