Cardiovascular adaptations to transfusion/chelation therapy of homozygote sickle cell anemia.

The effect of transfusion/chelation therapy on the cardiovascular adaptations to chronic anemia in pediatric and young adult patients with homozygous sickle cell disease is uncertain. This study compares left ventricular (LV) function indexes and thoracoabdominal aortic systolic and diastolic blood flow in nontransfused and transfused patients with homozygous sickle cell disease. The study population consisted of 29 nontransfused patients with homozygous sickle cell disease, ages 0.4 to 20.9 years (group 1) and 11 chronically transfused/chelated patients, ages 4.0 to 21.8 years (group 2). The mean total hemoglobin concentration in group 2 was 28% greater than that in group 1. The mean duration of transfusion/chelation therapy in group 2 was 3.7 years. The percent of predicted LV end-diastolic and end-systolic dimensions were significantly greater than the respective controls in both groups. There was no significant difference in percent of predicted LV end-diastolic dimension (group 1, 120 +/- 12%; group 2, 120 +/- 12%) or percent of predicted LV end-systolic dimension (group 1, 120 +/- 12%; group 2, 117 +/- 8) between the groups. The percent of LV shortening fraction was similar in study groups and control subjects. Aortic systolic blood flow (cc/min/m2) for group 1 (2,426 +/- 841) and 2 (2,374 +/- 1.004) were significantly greater than corresponding control values (1,683 +/- 442, 1,736 +/- 430, respectively). Aortic diastolic blood flow was significantly greater than corresponding control values for both group 1 (699 +/- 313 vs 488 +/- 212) and group 2 (1,080 +/- 607 vs 588 +/- 219).(ABSTRACT TRUNCATED AT 250 WORDS)