Right ventricular function among South East Nigeria children with sickle cell anaemia
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RESEARCH ARTICLE
Open Access
Right ventricular function among South East Nigeria children with sickle cell anaemia Josephat M. Chinawa1, Bartholomew F. Chukwu1* , Awoere T. Chinawa2, Edmund N. Ossai3, Anthony N. Ikefuna1, Ann E. Aronu1 and Egbuna O. Obidike1
Abstract Background: Sickle cell anaemia (SCA) is characterized by attendant ischemia-reperfusion injury especially to the heart. Methods: The 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. 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%), {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%. Conclusion: Children 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. Keywords: Anaemia, Children, Enugu, Sickle cell anaemia, Right ventricular function
Background Sickle cell anemia is a hematologic and genetic disease characterized by recurrent episodes of ischemiareperfusion injury to multiple vital organ systems [1]. Though volume overload is noted as a cause of cardiac abnormalities in SCA, other potential causes are * Correspondence: [email protected] 1 Department of Paediatrics, College of Medicine, University of Nigeria Enugu Campus, Enugu, Nigeria Full list of author information is available at the end of the article
ischemia-reperfusion injury and iron overload toxicity in chronically transfused patients [2]. It is important to note that the underlying haemolytic anaemia in children with sickle cell anaemia increases cell-free plasma haemoglobin which depletes nitric oxide and causes vasoconstriction, leading to pulmonary hypertension [2]. Both systolic and diastolic dysfunction of the right ventricle coupled with right ventricular hypertension had been documented in sickle cell anaemia at age 3 years [2]. They are due to progressive increase in pulmonary
© The Author(s). 2020 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use,
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