195 Echocardiographic evaluation in children with sickle cell anemia

Background Sickle cell anemia (SCA) is a hemoglobinopathy that is common worldwide. Cardiac abnormalities in sickle cell anemia are frequent and early. However, they are not well investigated among children. The aim of our study was to evaluate the cardiac function in children with sickle cell anemia compared to echocardiographic parameters in controls. Left ventricular two-dimensional strain was assessed as an early marker of systolic dysfunction. Methods The study was directed in the echocardiographic laboratory in the military hospital of Tunis between July 2018 and December 2018. Thirty patients with SCA and fifty-eight controls matched for age and sex without SCA or heart disease, were compared. The echocardiographic measurements were indexed according to body surface. We have studied several morphological and functional parameters using echocardiography. Cardiac deformability of the left and the right ventricles was studied using two-dimensional longitudinal strain. Results Our study population was divided into two matched groups. The first group included 30 patients (11.8 ± 2 yrs, sex ratio: 1.31) with homozygous SCA and the second group included 30 healthy controls (12.7 ± 1,2 yrs, sex ratio: 1.27). According to the findings, the first group showed significantly larger LV diameter (36.2±2.5 mm/m2 vs 29.3±1.3 mm/m2, p=0.005). We found also a left ventricular hypertrophy in patients with SCA. The left ventricular mass was of 109±22,05 g in patients and 69±25,6 g in controls. The first group showed lower LV ejection fraction (62%±0.5 vs 65%±5, p=0.001). The study of systolic function of the right ventricle did not show a significant difference between the two groups. In addition, we reported an elevation of PAPS in the sickle cell group compared to controls (30.7 ± 4.2 mmHg, versus 24.2 ± 5.9 mmHg for controls, p Conclusion Cardiac abnormalities began early in childhood and progressively increase with age. Our study highlights several cardiac abnormalities in children with SCA, which could represent a marker of disease severity. Elevated LVGLS might indicate an early compensatory mechanism in response to upstream myocardial insults and may serve as an early marker of altered myocardial function in children with SCA. Conflict of Interest no