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Published on: March 14, 2017
Cardiac abnormalities in children with sickle cell anemia
L A Lester1, P C Sodt, N Hutcheon
1Department of Pediatrics, University of Chicago Pritzker School of Medicine.
Insights
Children with sickle cell anemia show enlarged heart chambers due to anemia-related volume overload. Echocardiography reveals no distinct sickle cell cardiomyopathy in this pediatric group.
Area of Science:
- Pediatric Cardiology
- Hematology
- Cardiovascular Research
Background:
- Sickle cell anemia (SCA) is a genetic blood disorder.
- Cardiac complications are a concern in SCA patients.
- Understanding cardiac status in pediatric SCA is crucial.
Purpose of the Study:
- To evaluate the cardiac status in children with sickle cell anemia.
- To compare cardiac dimensions and function with healthy controls.
- To investigate the relationship between cardiac abnormalities and anemia severity.
Main Methods:
- Echocardiography was used to assess cardiac status in 64 children with SCA.
- Cardiac dimensions (left atrial, left ventricular, aortic root) and wall thickness were measured.
- Multivariate regression analysis correlated cardiac findings with hemoglobin levels and Hemoglobin S percentage.
Main Results:
- Over 60% of children with SCA exhibited enlarged left atrial, left ventricular, and aortic root dimensions compared to controls.
- Increased left ventricular mass and cardiac index were observed in SCA patients.
- Cardiac abnormalities correlated significantly with anemia severity (hemoglobin levels) and Hemoglobin S percentage.
Conclusions:
- The primary cardiac abnormalities in pediatric SCA are linked to chronic anemia-induced volume overload.
- No evidence of a distinct sickle cell cardiomyopathy or cardiac dysfunction was found in this pediatric cohort.
- Echocardiographic assessment revealed normal resting left ventricular function in SCA children.
Abstract:
The cardiac status of 64 children (ages 0.2 to 18 yr) with sickle cell anemia documented by hemoglobin electrophoresis was evaluated by echocardiography. Left atrial, left ventricular and aortic root dimensions were significantly increased in over 60 percent of these children at all ages compared to values for 99 normal black (non-SCA) control subjects. Left ventricular wall thickness was increased in only 20 percent of older children with sickle cell anemia. Estimated LV mass/m2 and left ventricular cardiac index were increased compared to control subjects (p less than 0.001). Left heart abnormalities expressed as a single composite function, derived from multivariate regression analysis, correlated well with severity of anemia expressed as grams of hemoglobin (r = -0.52, p = less than 0.001) and with percentage of hemoglobin S (r = 0.51, p less than 0.001), but not to the same extent with age. Echocardiographically assessed left ventricular function at rest was comparable to that of control subjects. These data suggest that the major cardiac abnormalities in children are related to the volume overload effects of chronic anemia, and that in this age group, there is no evidence for a distinct "sickle cell cardiomyopathy" or cardiac dysfunction.
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