Left Ventricular Geometric Patterns and Systolic Myocardial Performance in Nigerian Children with Homozygous Sickle Cell Anaemia

Background: Cardiac complications contribute significantly to morbidity in children with sickle cell anaemia (SCA). Little is known about the geometry and contractile function of the left ventricle (LV) of affected children in sub-Saharan Africa, which has the greatest burden of thisdisease. Objective: To compare the LV systolic function of children with SCA in the steady state with that of matched haemoglobin AA controls. Methods: Clinical, laboratory and LV M-mode echocardiographic parameters of 120 steady-state SCA patients aged 3-14 years were compared with those of matched Hb AA controls. Univariate and multivariate analyses were performed using SPSS software, version 22 (IBM, Armonk, NY, USA). Results: The median ejection fraction of 62.00 (IQR=55.0-67.0) for the subjects was significantly less than that of 68.00 (IQR=65.8-73.0) for the controls (p< 0.001) and correlated negatively with age (ρ=-0.25, p= 0.006), BSA (ρ=-0.24, p= 0.008), systolic BP (ρ=-0.23, p=0.022), and diastolic BP (ρ=-0.31, p=0.002). Left ventricular systolic dysfunction (LVSD) was present in 27% of the SCA patients but only in 1% of the controls (χ² = 26.5, p< 0.001). The odds of having LVSD decreased by 0.9 for every 1% increase in HbF level (CI= 0.82-0.99, p= 0.03). Abnormal LV geometry, detected in 52% of the subjects, was associated with the presence of LVSD (Fisher’s exact test, p< 0.001). The odds of having abnormal LV geometry decreased by 0.5 for every 1 g increase in Hb level (CI=0.27-0.87, p=0.02). Conclusions: LVSD is more common in SCA patients than in controls and is less likely to occur in patients with higher HbF levels. Approximately one in every two children with SCA have abnormal LV geometry, which is less likely to occur at higher Hb levels.