Vascular endothelial growth factor and pulmonary hypertension in children with beta thalassemia major.

OBJECTIVE: The purpose of this study was to illustrate the association between vascular endothelial growth factor level and pulmonary artery hypertension in children with ß-thalassemia major. METHOD: This case-control study was conducted on 116 children with ß-thalassemia major; 58 of them had pulmonary artery hypertension. They were compared to 58 healthy children who were age and sex-matched (control group). Serum levels of vascular endothelial growth factor and echocardiographic assessment were done for all children. RESULTS: Vascular endothelial growth factor serum level was significantly higher in children with ß-thalassemia major with pulmonary artery hypertension than in those without pulmonary artery hypertension, as well as in control groups (p<0.001). Vascular endothelial growth factor serum level had a significant positive correlation with pulmonary artery pressure and serum ferritin, as well as a significant negative correlation with the duration of chelation therapy. Logistic regression analysis revealed that elevated vascular endothelial growth factor (Odd Ratio=1.5; 95% Confidence Interval, 1.137-2.065; p=0.005) was an independent risk factor of pulmonary artery hypertension in such children. Vascular endothelial growth factor serum level at a cutoff point of >169pg/mL had 93.1% sensitivity and 93.1% specificity for the presence of pulmonary artery hypertension in children with ß-thalassemia major. CONCLUSION: Elevated vascular endothelial growth factor serum level is associated with pulmonary artery hypertension in children with ß-thalassemia.