Right ventricular function among South East Nigeria children with sickle cell anaemia

Josephat M Chinawa,Anthony N Ikefuna,Ann E Aronu,Edmund N Ossai,Awoere T Chinawa,Egbuna O Obidike,Bartholomew F Chukwu

doi:10.1186/s12887-020-02143-4

Abstract

BackgroundSickle cell anaemia (SCA) is characterized by attendant ischemia-reperfusion injury especially to the heart.MethodsThe aim of this work is to compare the right ventricular function of children with SCA in steady state (subjects) with those with haemoglobin AA genotype (controls), using echocardiography. It is a cross-sectional study, which echocardiographic measurements to assess right ventricular function among children with SCA and their controls.ResultsThe 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%), {χ2 = 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, (χ2 = 11.668, p = 0.001). The prevalence of right ventricular diastolic dysfunction in subjects was 9.8% while control was 0%.ConclusionChildren with sickle cell anaemia present with right ventricular dysfunction. Prevalence of right ventricular systolic and diastolic dysfunction were higher in subjects. More of the subjects in this study (25.5%) had pulmonary hypertension.

Highlights

Sickle cell anaemia (SCA) is characterized by attendant ischemia-reperfusion injury especially to the heart
When we looked at the overall prevalence rate of right diastolic dysfunction for both subjects and controls, we noted that children with sickle cell anaemia had a prevalence rate of 9.8% compared with their normal counterparts who had 0% prevalence
The mean Trans annular plane systolic excursion (TAPSE) of children with sickle cell anaemia was significantly higher than that obtained in children with haemoglobin AA genotype

Summary

Introduction

Sickle cell anaemia (SCA) is characterized by attendant ischemia-reperfusion injury especially to the heart. Sickle cell anemia is a hematologic and genetic disease characterized by recurrent episodes of ischemiareperfusion injury to multiple vital organ systems [1]. Elevated tricuspid regurgitation velocity (TRV), TAPSE and Tricuspid inflow (e/a) velocity (TV E/A) which are surrogate markers for pulmonary hypertension (PHT) are very relevant indices of right ventricular dysfunction which occurs in children with sickle cell anaemia and is associated with low hemoglobin and elevated reticulocyte count [5]. Accurate assessment of right ventricular function of children with sickle cell anaemia in steady state by echocardiography helps in early detection of cardiac diseases, enhances risk stratification, and allows timely initiation of appropriate therapy [6] The cause of right ventricular dysfunction in children stems mainly from progressive increase in pulmonary resistance, whereas in adults; it is usually from high steady-state serum lactate dehydrogenase (LDH) levels, largely reflecting intravascular haemolysis, renal insufficiency, cholestatic hepatic dysfunction and iron overload [3].

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