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Coronary artery dilation in sickle cell disease
George T Nicholson1, Daphne T Hsu, Steven D Colan
1Department of Pediatrics, Children's Hospital at Montefiore, Bronx, NY, USA. gnichols@montefiore.org
The Journal of Pediatrics
|July 5, 2011
Summary
Coronary artery ectasia (CAE) affects 17.7% of children with sickle cell disease (SCD). This condition is linked to heart muscle thickening and inflammation in these patients.
Area of Science:
- Pediatric Cardiology
- Hematology
- Cardiovascular Research
Background:
- Sickle cell disease (SCD) is a genetic blood disorder with known cardiovascular complications.
- Coronary artery abnormalities are increasingly recognized in pediatric populations.
- Understanding the prevalence and associations of coronary artery dilation in SCD is crucial for risk stratification.
Purpose of the Study:
- To determine the prevalence of coronary artery ectasia (CAE) in pediatric patients with sickle cell disease (SCD).
- To identify clinical and echocardiographic factors associated with CAE in this population.
Main Methods:
- Retrospective analysis of 96 patients aged 10-19 years with SCD undergoing echocardiography.
- Coronary artery diameters were measured and converted to z scores using a reference population.
- Coronary artery ectasia defined as a z score ≥ 2; comparison of patients with and without CAE using univariate and multivariate analyses.
Main Results:
- Coronary artery ectasia (CAE) was identified in 17.7% (17 of 96) of children with SCD.
- Patients with CAE showed increased left ventricular dimensions, mass-to-volume ratio, and white blood cell count.
- Multivariate analysis indicated that mass-to-volume ratio and elevated white blood cell count were significantly associated with CAE.
Conclusions:
- Coronary artery ectasia (CAE) is a common finding in children with sickle cell disease (SCD).
- CAE in SCD is associated with indicators of left ventricular hypertrophy and systemic inflammation.
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