Assessment of Right Ventricular Function in Children with Sickle Cell Anaemia Seen in Enugu, Nigeria.

Abstract Background: Sickle cell anaemia (SCA) is characterized by attendant ischemia-reperfusion injury especially to the heart. Objectives: To compare the right ventricular function of children with SCA in steady state (subjects) with those with haemoglobin AA genotype (controls), using echocardiography. Methods: The cross-sectional study utilized echocardiographic measurements to assess right ventricular function among children with SCA and their controls. Results: The mean trans annular plane systolic excursion (TAPSE) in subjects, 28.24±5.23 (Z score: 0.258±1.10) was higher than that in control, 25.82±3.59 (Z score: - 0.263±0.80), and the difference in mean was statistically significant, (t=2.703, p=0.008). Significantly higher proportion of subjects with sickle cell anaemia had right ventricular dysfunction (Abnormal TAPSE), 25 (50.0% when compared with those in control, 11 (22.0%, (χ 2 =8.5, p=0.0035). A higher proportion of subjects with sickle cell anaemia (25.5%) had Pulmonary hypertension (RVP) when compared with control (2.0%) and the difference in proportions was found to be statistically significant, (χ 2 =11.668, p=0.001). The prevalence of right ventricular diastolic dysfunction in subjects was 9.8 % while control was 0%. Conclusion Children with sickle cell anaemia present with right ventricular dysfunction. Prevalence of right ventricular systolic and diastolic dysfunction were higher in subjects. More children with genotype SS group, (25.5%) had pulmonary hypertension (RVP). Key words: Anaemia; children; Enugu; sickle cell anaemia; right ventricular function