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Cardiac abnormalities in children with sickle cell anemia
Anjan S Batra1, Ruben J Acherman, Wing-yen Wong
1Division of Cardiology, Children's Hospital of Los Angeles and LAC+USC Medical Center, Los Angeles, California, USA. abatra@iupui.edu
American Journal of Hematology
|September 5, 2002
Summary
Sickle cell anemia (SCA) causes heart volume overload. Despite this, systolic function is preserved, but chronic transfusions may lead to diastolic dysfunction in children.
Area of Science:
- Pediatric Cardiology
- Hematology
- Cardiovascular Physiology
Background:
- Sickle cell anemia (SCA) leads to chronic heart volume overload due to hemodilution.
- Previous cardiac studies in SCA children often overlooked abnormal loading conditions.
Purpose of the Study:
- To correlate anemia severity and transfusion status with cardiac findings in SCA.
- To assess cardiac function using load-independent parameters in pediatric SCA patients.
Main Methods:
- Echocardiography, physical examination, and ECG were used in 77 SCA patients (ages 2-22).
- Patients were divided into non-transfused (n=57) and chronically transfused (n=20) groups.
Main Results:
- Non-transfused patients had lower hemoglobin, higher cardiac output, and larger left ventricular (LV) dimensions and mass.
- Load-independent systolic function (velocity of circumferential fiber shortening-wall stress index) was preserved in both groups.
- LV myocardial performance index, indicating combined systolic/diastolic function, was higher in transfused patients, suggesting diastolic dysfunction.
Conclusions:
- SCA causes a volume-overloaded heart with increased LV size and mass, proportional to anemia severity.
- Systolic cardiac function remains preserved despite abnormal loading conditions in SCA.
- Chronic transfusions in SCA may be associated with impaired diastolic function, even with iron chelation.