Cardiac aspect of Cooley's anemia.

The short- and long-term effect of multiple versus regularly spaced blood transfusions on cardiac size, electrocardiographic alterations, heart sounds, murmurs, and carotid pulse were studied in 18 children with Cooley's anemia. These parameters were evaluated before transfusions, one week after multiple blood transfusions (when hemoglobin levels had reached normal), and during the following year, when conventionally spaced transfusions were given. Results showed that enlarged hearts may regress to normal size after multiple transfusions. Chamber hypertrophies and second heart sound were inconstant, and the average of all mean QRS and T vectors was not altered significantly after transfusions. There was good correlation between hemoglobin levels and tachycardia, intensity of first heart sound, heart murmurs, and ejection systolic clicks. Upstroke time of carotid pulse increased with restoration of normal hemoglobin levels. It is concluded that patients maintained at a higher hemoglobin level are generally in better cardiovascular status.