ABSTRACT
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. Anatomical and functional assessment of the heart through M mode, 2 dimension and doppler echocardiography in pediatric patients with sickle cell anemia [SCA]. Twenty-five children with SCA [mean age 10 +/- 3.4 years], 14 males and 11 females were prospectively studied in a comparison with 25 ages and sex matched healthy children. All of them underwent clinical and laboratory evaluation, M mode, 2 dimension and doppler echocardiography. Patients with SCD had higher left ventricular end diastolic dimension, left atrial dimension, stroke volume and left ventricular mass. Mild pulmonary hypertension with mean pulmonary artery systolic pressure [PASP] 30 +/- 5.40 mmHg. Chamber dilatation, 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