Left ventricular performance in children with homozygous sickle cell anaemia.

Abstract

Left ventricular performance was determined by echocardiography in 44 black children with homozygous sickle cell anemia and a control group of 28 normal black children of comparable age. Statistically significant differences were observed between the children with sickle cell anemia and the normal group in left ventricular ejection fraction (sickle cell anemia group: 0.59 .+-. 0.01 [mean .+-. standard error of the mean] vs. normal group: 0.65 .+-. 0.01), cardiac index (5.3 .+-. 0.3 vs. 4.2 .+-. 0.3 l/min per m2), mean circumferential fiber shortening velocity (1.16 .+-. 0.04 vs. 1.31 .+-. 0.03 s-1) and the percentage of shortening of left ventricular minor axis dimension (32.5 .+-. 1 vs. 36.7 .+-. 0.8). The children with sickle cell anemia were divided into 2 groups according to the absence or presence of dyspnea and/or fatigue on moderate effort; though both groups had the same degree of anemia, significantly depressed left ventricular performance indices were observed only in the group of symptomatic patients. All asymptomatic children with sickle-cell anemia had uncompromised left ventricular performance. Left ventricular dysfunction apparently is present in a significant proportion of children with sickle cell anemia. The extent of the left ventricular dysfunction is not related to the degree of anemia or the percentage of fetal hemoglobin. Since many of the symptoms, physical signs and radiological findings of severe anemia resemble those of congestive heart failure, echocardiographic examination of symptomatic children with homozygous sickle cell anemia is useful in detecting the presence of left ventricular dysfunction.