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Background: Secondary pulmonary hypertension (PH) is emerging as a significant cause of mortality and morbidity in patients with thalassemia. Tricuspid Regurgitant Jet Velocity (TRJV) can identify patients at risk for PH. Aim of the work: To study the effect of the L-arginine and Sildenafil on patients with β -thalassemia complicated by increased Tricuspid Regurgitant Jet Velocity. Patients and methods: A randomized controlled clinical trial was carried out on 60 children with β-thalassemia major, selected with high TRJV (TRJV>2.5 m/s). The children were selected from Hematology Unit, Pediatric Departments, Tanta University Hospital. They were classified into 3 groups: Group (I): Twenty children received L-arginine, group (II): Twenty children received Sildenafil therapy and group (III): Twenty children did not receive either L-arginine or sildenafil therapy as a control group. All Patients were matched in ethnicity, age and sex distribution. They underwent to full history taking and complete clinical examination. Investigations include; complete blood count, aspartate transaminase (AST), alanine transaminase (ALT), serum ferritin and Color Doppler Echocardiography to measure TRJV. Results: There was no statistically significant difference between three groups as regard platelet count, serum ferritin, serum ALT, AST. There was a significant decrease of TRJV after Sildenafil therapy and/or L-arginine therapy, with no statistically significant differences between group (I) and group (II) as regard to TRJV after treatment. Conclusion: Pediatric patients with thalassemia complicated by PH can get benefit from therapy with Sildenafil and/or arginine therapy.