Sickle cell heart disease. Two-dimensional echo and Doppler ultrasonographic findings in the hearts of adult patients with sickle cell anemia.

Sickle cell anemia (SCA) is a commonly diagnosed condition in the black population in the United States. With better medical management, patients with SCA are living longer and what were previously uncommon sequelae are being recognized frequently, including those involving the cardiovascular system. Previous reports on the cardiovascular effects of SCA have focused predominantly on children, but the conclusions remain controversial. To study this question further, 40 adult patients with documented SCA were examined using two-dimensional and Doppler cardiac ultrasound. Valvular structures were normal despite an increased incidence of flow murmurs. Abnormalities were found more frequently in the left heart than the right, as manifested primarily by increased left ventricular mass, and left ventricular and left atrial dilatation with preservation of systolic function. Pulmonary hypertension, which was present in two thirds of the sample, was minimal to moderate as assessed by our Doppler technique. The effects of SCA on the heart seem to be minimal and similar to those of other anemias, predominantly confined to the left atrium and ventricle with passive elevation of pulmonary pressures. Clinical murmurs were most often physiologic; there was no association with myxomatous valvular degeneration or mitral valve prolapse.