Objective: To assess the prevalence and the risk factors of pulmonary hypertension in children with sickle cell disease. Methods: Children (age: 3- 15 y) diagnosed with sickle cell disease were included in the study conducted between August 2016 to July 2018. All relevant past history of hospitalisation, crisis (esp. acute chest syndrome crisis), blood transfusion and medication history was recorded. A thorough physical examination, blood investigations (CBC and Reticulocyte Count) and 2D Echocardiography was carried out for all the enrolled patients. Results: In this study, 18.57% of the study population was found to have PAH with TRV > 2.5 m/s. No statistically significant co-relation was found between age, gender, religion, anthropometry, blood group type, sickle phenotype, total leukocyte count and PAH. There was a statistically significant association of pulmonary hypertension with low Hb, high reticulocyte count and platelet count, history of repeated hospital admissions and blood transfusions and a positive history of ACS.It was also found that patients who were on hydroxyurea treatment were less likely to develop PAH. Conclusion: One-fourth of pediatric sickle cell disease patients demonstrated presence of pulmonary hypertension. Risk factors like low Hb, elevated platelet and reticulocyte count, repeated number of hospital admissions, episodes of ACS and blood transfusions were significantly associated with an elevated TRV finding on Echocardiography in children with Sickle Cell Disease. Hydroxyurea therapy too played a significant role in this study in reducing the chances of having PAH. These patients need to be monitored and followed-up after treatment to assess need for later treatment

Keywords: Sickle Cell Disease; Pulmonary Hypertension; Doppler Echocardiography; Tricuspid Regurtiant Velocity