Cardiac performance in children with homozygous sickle cell disease

Cardiac function was evaluated in 24 children from a Jamaican sickle cell cohort study. Ten patients with sickle cell disease underwent echocardiographic studies on their eighth birthday. The results were compared with 14 age- and sex-matched control children born within hours of the index patients. Left ventricular dimension index (systolic 2.89 +/- 0.31 versus 2.33 +/- 0.42 cm and diastolic 4.70 +/- 0.35 versus 3.64 +/- 0.48 cm, p = 0.001), diastolic volume (79.4 +/- 17.1 versus 60.8 +/- 7.8 ml, p = 0.01), left ventricular mass index (116.3 +/- 3.4 versus 74.3 +/- 15.2 g/m2, p = 0.001) and cardiac index (5.51 +/- 1.32 versus 3.38 +/- 0.85 liters/min per m2 p = 0.001) were significantly increased in patients with sickle cell disease compared with values in control subjects. However, there was no statistically significant difference between the two groups for ejection fraction, velocity of circumferential fiber shortening, percent fractional shortening, systolic time intervals, wall stress and ratio of wall stress-systolic volume. Although two mean ratios of wall stress-systolic volume index were lower in children with sickle cell disease as compared with control subjects (4.0 +/- 0.7 versus 5.4 +/- 1.7, p = 0.02 and 5.9 +/- 1.2 versus 8.3 +/- 2.5, p = 0.005, respectively), the range of ratios remained within normal limits (3.4 to 5.8 in children with sickle cell disease versus 2.8 to 9.5 in controls and 4.2 to 8.3 versus 3.8 to 12.5, respectively). Furthermore, only body surface area predicted group status independent of other variables (p = 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)