Left ventricular performance in children with homozygous sickle cell anaemia.

Abstract

SUMMARY 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.Children and adults with homozygous sickle cell with the tendency of the sickle cells to occlude small anaemia frequently present evidence of cardio-vessels in the systemic, pulmonary (Moser et al., vascular system abnormalities (Klinefelter, 1942;1960), and coronary circulation (Oliveira and

Right ventricular abnormalities in sickle cell anemia: Evidence of a progressive increase in pulmonary vascular resistance

“…The cardiac effects of this chronic illness on the left ventricle have been extensively investigated in both adults [1][2][3][4][5] and children. [6][7][8][9][10][11][12][13] However, fewer adult studies, 4 -5,14 -17 only 1 study that included adolescents, 11 and no studies involving children have investigated any aspect of the impact of SCA on the right ventricle (RV). It was recently reported that 32% of young adults with SCA have pulmonary hypertension, an important predictor for premature death.…”

Myocardial ischaemia in children with sickle cell disease

“…Twenty two children met one or more of these criteria. Twelve were male and 10 female; mean age was 11.24 (1.04) years (range [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19]. Twenty one had homozygous sickle cell disease (SS), one was S/b 0 -thalassemic.…”

Left ventricular systolic function in sickle cell anaemia: An echocardiographic evaluation in adult Nigerian patients

“…Left ventricular performance determined by echocardiography in 44 black children with sickle cell anaemia and a control group of 28 normal black children of comparable age by Rees et al 5 revealed statistically significant differences between the children with sickle cell anaemia and the normal group in left ventricular ejection fraction, cardiac index, mean circumferential fiber shortening velocity, and the percentage of shortening of left ventricular minor axis dimension. These findings consistent with left ventricular systolic function have been corroborated by the report of Balfour and colleagues 6 who also observed that left ventricular function became increasingly abnormal with age, suggesting that left ventricular function deteriorated with time.…”

“…These measures are highly dependent on and influenced by myocardial loading conditions as well as heart rate, both of which are abnormal in sickle cell anaemia 3,4 . Use of these indices of systolic function in this population may explain the broad spectrum of results and apparent inconsistencies documented in the literature 5,6,7,8,9 .…”