Pulmonary Hypertension and β-Thalassemia Major: Report of a Case, Its Treatment, and a Review of the Literature


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Abstract

Pulmonary hypertension is a common complication of β-thalassemia major. We report a case of successful treatment of pulmonary hypertension in a patient with β-thalassemia major and review the literature on pulmonary hypertension and β-thalassemia major. A 28-year-old man with β-thalassemia major, splenectomy, hepatitis C, and hemosiderosis who presented with increasing dyspnea on exertion was diagnosed with pulmonary hypertension. After receiving continuous epoprostenol infusion and desferoxamine, his functional capacity and hemodynamic status improved. To our knowledge, this is the first case of pulmonary hypertension associated with β-thalassemia treated with continuous epoprostenol infusion and desferoxamine. Epoprostenol, beneficial in the treatment of other types of pulmonary hypertension, may ameliorate the morbidity and mortality of pulmonary hypertension associated with thalassemia.

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