Predictive factors associated with subclinical altered left ventricular function in children with sickle cell anemia

Abstract

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 left ventricular (LV) function in children with sickle cell anemia. We conducted a study in the echocardiographic laboratory in the military hospital of Tunis between July 2019 and December 2019. 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. Our study population was divided into two matched groups: Group 1: included 30 patients with homozygous SCA. Group 2: included 30 healthy controls. Group 1 showed significantly larger LV diameter (36.2 ± 2.5 mm/m2 vs. 29.3 ± 1.3 mm/m2, P = 0.005). Group 1 also showed lower LV ejection fraction (62% ± 0.5 vs. 65% ± 5, P = 0.001). Whereas, no significant difference was observed for cardiac output (P = 0.4). Otherwise, two-dimensional longitudinal strain of LV was higher in SCA group (−21% ± 3.07 vs. −25% ± 2.98; P < 0.01). We found a P-value within the limit of significance between the occurrence of certain complications and the alteration of SGL such as the occurrence of osteonecrosis (P = 0.053, R = 0.44), splenomegaly (P = 0.051, R = 0.61), splenic sequestration (P = 0.074, R = 0.34) and dyspnea (P = 0.075, R = 0.38). The influence of hydroxycarbamine treatment on alteration of SGL was close to significance (P = 0.075, R = −0.07). We found a statistically significant influence between the alteration of SGL and the dosage of hydroxycarbamine (P = 0.043, R = −0.46). Our study highlights several cardiac abnormalities in children with SCA, which could represent a marker of disease severity. Our study suggests that hydroxycarbamine treatment may attenuate the alteration of myocardial function in children with SCA.