Pulmonary arterial hypertension in children with sickle cell anaemia

Abstract

Background:Pulmonary hypertension(PHT) is a life threatening complication which occurs in adults with sickle cell disease(SCD). But there is paucity of knowledge about prevalence of pulmonary hypertension in Paediatric SCD population.Objective:To find out the prevalence of pulmonary hypertension in children with SCD.Methods:552 children age between 5 to 14 years were enrolled after written informed consent was obtained from parents/guardian. Children with Congenital heart disease, pulmonary stenosis, Rheumatic heart disease were excluded from the study. PHT in all cases of with SCD children was screened by using 2D Echocardiography in the department of cardiology. PHT is defined as mean pulmonary artery pressure (mPAP) ≥ 25 mmHg at rest. All the data were validated and analyzed with SPSS version 25.Results:Overall prevalence of pulmonary hypertension in our study is 20.4%. Of these, 6.5% subjects have mild and 13.9% subjects have moderate pulmonary hypertension. Low haemoglobin (p=0.021), high number of crisis (p=0.000), high number of blood transfusion (p=0.000), presence of loud second heart sound (p=0.000) and presence of Parasternal heave (p=0.000) were significantly associated with pulmonary hypertension. The prevalence of pulmonary hypertension in children with SCD demonstrated an increase in trend with age.Conclusion:High pulmonary artery pressures do occur in children with SCD. Screening by echocardiography can lead to early detection and intervention.