Abstract

Background: Sickle cell anaemia (SCA) is the most common and burdensome inherited disease affecting children in Africa. Nonetheless, there remains a paucity of literature from Nigeria about its effects on cardiac dimensions and functioning in children. Objective: To determine cardiac dimensions and left ventricular (LV) systolic function in children with SCA in steady state and compare with same in healthy haemoglobin type AA controls. Methodology: Case-controlled, cross-sectional study of 50 subjects aged ≤ 15 years and 50 age- and sexmatched controls to determine and compare aortic root diameter (Ao), left atrium diameters (LAD), LV dimensions and indices of LV function [fractional shortening (FS) and ejection fraction (EF)] using 2-D guided M-mode echocardiography. Body surface areas (BSA) and venous haematocrits were also assessed. Results: Subjects had significantly higher mean Ao and LAD than controls (p=0.020 and p<0.001 respectively). The LV dimension indices measured were all significantly higher in subjects, excepting LV-posterior wall diameter in diastole. Almost half (44.0%) of the subjects had LV hypertrophy and almost all (95.5%) were eccentric. Nevertheless, subjects and controls had similar systolic cardiac function [FS (%); 32.04 vs. 32.29, p=0.871 and EF (%); 59.94 vs. 60.86, p=0.326] and these showed no correlation with age, BSA or haematocrit in both study groups. Conclusion: Nigerian children with SCA have deviations in cardiac dimensions with enlargements in various echocardiographic indices ranging from 1.1 to 1.4 times those of normal children. Yet, these children retain relatively normal systolic cardiac function.

Highlights

  • Sickle cell anaemia (SCA), a disease of public health significance affects millions of people globally and remains the most common, severe inherited disease in Africa [1]

  • There is no consensus on the cardiovascular function of persons with SCA as the medical literature abounds with conflicting reports

  • Some echocardiography (ECHO) studies report normal left ventricular (LV) function in spite of increase in cardiac size and mass noted [6,9] while others report LV dysfunction in children with SCA [8,10]

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Summary

Introduction

Sickle cell anaemia (SCA), a disease of public health significance affects millions of people globally and remains the most common, severe inherited disease in Africa [1]. It is associated with multiple structural and organ dysfunctions including potentially lifethreatening cardiovascular complications [2]. Some echocardiography (ECHO) studies report normal left ventricular (LV) function in spite of increase in cardiac size and mass noted [6,9] while others report LV dysfunction in children with SCA [8,10]. Sickle cell anaemia (SCA) is the most common and burdensome inherited disease affecting children in Africa. There remains a paucity of literature from Nigeria about its effects on cardiac dimensions and functioning in children

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