Treatment of pulmonary hypertension in three patients with β-thalassemia intermedia using pulmonary arterial hypertension-specific medications

Pulmonary hypertension (PH) is frequent among patients with β-thalassemia intermedia (TI) and β-thalassemia major (TM) (1). Almost 60% of all TI patients develop PH (2). However, no randomized controlled trials have evaluated this condition-specific treatment options. Recent guidelines for the treatment of PH offer no specific recommendations for these patients; moreover, the classification of chronic hemolytic anemia was changed from group I PH to group V PH, in which group pulmonary arterial hypertension (PAH)-specific therapy is not recommended (3). We report three patients with β-TI who developed severe PH and were successfully treated with PAH-specific therapies.