Pulmonary hypertension in a patient with beta-thalassemia major

We report a case of pulmonary hypertension (PH) in a 35-year old patient wi th beta-thalassemia major, he had commenced blood transfusions after the ag e of 4 years and had been splenectomised at the age of 6 yeats. PH clinical presentation was riot uncommon. Hemodynamic study revealed precapillary PH with high cardiac output; vasodilators agents led to significant pulmonary responsiveness. In beta-thalassemia, whereas congestive heart failure is common and due to cardiac hemosiderin deposition, PH appears to be non rare but its etiopatho genic mechanism remain unclear and probably non univoqual. Hypoxemia as wel l as hemodynamic changes related to chronic anemia including increased pulm onary flow might play an important role. Management should include blood tr ansfusions to correct anemia, the indication and the choice of vasodilator agents need to be evaluated.