Noninvasive Assessment of Left Ventricular Size and Function in Infants and Young Children with Sickle Cell Anemia

The authors evaluate the effects of sickle cell anemia in infancy, including increased heart rate and left ventricular diameter. Thirty-three asymptomatic infants and young children were studied using M-mode echocardiography to determine left ventricular size and function. The data indi-cate that left ventricular diameter is larger than normal and that cardiac function (VCF) may be below normal. Key words: sickle cell anemia, left ventricular dimension, left ventricular function, echocardiography. Since sickle cell anemia was first described in 1910,1 the heart has been examined for effects of the disease. Enlarged heart, ECG abnormalities, and systolic mur-murs have been described.2 Autopsies have revealed hypertrophy and dilatation of the ventricles,3-4 which are more common in adults than in children.4 The heart, to compensate for the decreased hemo-globin associated with anemia, increases the volume of poorly oxygenated blood that is ejected from the left heart. Cardiomegaly results from the continued in-creased demand placed on the left ventricle.5 While there has been general agreement on the find-ings of left ventricular (LV) dilatation, there exists some disagreement as to the presence of LV dysfunc-tion. Gerry et al.s found no evidence of LV dysfunction; however, Rees et al.6 demonstrated LV dysfunction in children using echocardiography. In a recent study, Balfour et al.7 demonstrated increasing LV mass and progressive chamber enlarge-ment in children using echocardiography. The LV sys-tolic time interval ratio, a sensitive measure of cardiac function, increased in children with sickle cell anemia with growth at a rate beyond what was expected.8 This was due primarily to increases in the LV preejection period, which should be reduced in the presence of anemia