Prevalence of pulmonary hypertension in pediatric patients with beta-thalassemia major in Central India: a cross sectional study

Abstract

Background: The objective of the study was to determine the prevalence of pulmonary hypertension in pediatric patients with beta thalassemia major in a tertiary care centre in Central India.Methods: A cross sectional study was conducted in department of pediatrics, Government Medical College and Hospital, Nagpur, India over a period of 2 years. Total 100 subjects of thalassemia major diagnosed by high performance liquid chromatography (HPLC) attending day care in this department were enrolled for study. Measurement of pulmonary pressure was done by using 2D echocardiography with Doppler studies and tricuspid regurgitant jet velocity was measured. This was done by experienced cardiologist. Variables predictive of pulmonary hypertension studied were age, sex, serum ferritin levels, age of onset of chelation therapy, compliance to chelation therapy and number of blood transfusion per year.Results: Out of 100 subjects, 18 subjects had pulmonary hypertension. Univariate analysis proved that age, serum ferritin levels, age of onset of chelation therapy, compliance to chelation therapy and number of blood transfusion per year were significant predictors of pulmonary hypertension. While, multiple logistic regression analysis revealed that only 3 variables that is age of onset of chelation therapy, number of blood transfusion per year and compliance to chelation therapy retained their significance in this patient population.Conclusions: Prevalence of pulmonary hypertension was found out to be 18%. 2 subjects in the pulmonary hypertension group died. High morbidity and mortality of pulmonary hypertension clearly indicates that much work is needed in disease detection and development of more effective therapies. For thalassemic children, early detection and prevention of pulmonary hypertension in childhood would improve survival, reduce morbidity and mortality.