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Left ventricular performance in children with homozygous sickle cell anaemia.

Left ventricular performance was determined by echocardiography in 44 black children with homozygous sickle cell anaemia and a control group of 28 normal black children of comparable age. Statistically significant differences were observed between the children with sickle cell anaemia and the normal group in left ventricular ejection fraction (sickle cell anaemia 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 1/min per i2), mean circumferential fibre shortening velocity (116 + 0 04 vs 1-31 + 0.03s-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 anaemia were divided into two groups according to the absence or presence of dyspnoea and/or fatigue on moderate effort; though both groups had the same degree of anaemia, significantly depressed left ventricular performance indices were observed only in the group of symptomatic patients. All asymptomatic children with sickle-cell anaemia had uncompromised left ventricular performance. These findings indicate that left ventricular dysfunction is present in a significantproportionofchildren with sickle cell anaemia. The extent of the left ventricular dysfunction, is not related to the degree of anaemia or the percentage of fetal haemoglobin. Since many of the symptoms, physical signs, and radiological findings of severe anaemia resemble those ofcongestive heart failure, echocardiographic examination of symptomatic children with homozygous sickle cell anaemia is useful in detecting the presence of left ventricular dysfunction.