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Cardiac performance in children with homozygous sickle cell disease
Insights
Children with sickle cell disease show increased cardiac dimensions and mass, indicating cardiac remodeling. Body surface area is a key predictor of these changes in pediatric sickle cell patients.
Area of Science:
- Cardiology
- Hematology
- Pediatrics
Background:
- Sickle cell disease (SCD) is a genetic blood disorder with potential systemic complications.
- Cardiac involvement is a recognized, yet not fully understood, complication of SCD in children.
Purpose of the Study:
- To evaluate cardiac function in Jamaican children with sickle cell disease.
- To compare echocardiographic parameters between children with SCD and healthy controls.
Main Methods:
- Echocardiographic studies were performed on ten children with SCD on their eighth birthday.
- Results were compared with 14 age- and sex-matched control children.
Main Results:
- Children with SCD exhibited significantly increased left ventricular dimension index, diastolic volume, left ventricular mass index, and cardiac index compared to controls.
- No significant differences were found in ejection fraction, fractional shortening, or systolic time intervals.
- While some ratios of wall stress-systolic volume index were lower in SCD patients, they remained within normal limits.
Conclusions:
- Pediatric sickle cell disease is associated with cardiac remodeling, characterized by increased ventricular dimensions and mass.
- Body surface area independently predicted group status, suggesting its role in modulating cardiac changes in SCD.
Abstract:
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)