Assessment of cardiac functions in children with sickle cell disease

Abstract EN

Background: Sickle cell disease (SCD) is an inherited disorder associated with significant morbidity, characterized by the presence of abnormal hemoglobin within the red blood cells.

The cardiovascular system is stressed by chronic anemia, small pulmonary artery occlusions and myocardial hemosiderosis that cause multiple anatomical and functional changes. Aim : Anatomical and functional cardiac assessment through M mode, 2 dimension and Doppler echocardiography in pediatric patients with sickle cell anemia (SCA).

Methods: Forty children with SCA, 24 males and 16 females, of mean ages 10 ± 3.4 years admitted to Al jeddani hospital, Jeddah, Saudi Arabia with proven diagnosis of SCA were included in the study.

In addition, thirty age and sex matched healthy children were served as a control group.

All children were subjected to history taking, clinical examination and routine laboratory investigations (CBC, hemoglobin electrophoresis and serum ferritin) as well as M mode, 2 dimension and Doppler echocardiography. Results : patients with SCD had higher left ventricular end diastolic dimension, left atrial dimension, stroke volume and left ventricular mass compared to control group.

Mild pulmonary hypertension with mean pulmonary artery systolic pressure (PASP) of 30 ± 5.40 mmHg was observed in patients with SCD.

Conclusion: Chamber dilations, left ventricular hypertrophy with increased left ventricular mass associated with left ventricular diastolic dysfunction secondary to chronic anemia with volume overload and sickle cell cardiomyopathy confirm the evidence of the literature in characterizing a sickle cell disease in pediatrics.