Mortality in β-thalassemia patients with confirmed pulmonary arterial hypertension on right heart catheterization

Abstract

Pulmonary arterial hypertension (PAH) is a life-threatening complication of β-thalassemia, especially in untransfused patients with thalassemia intermedia. Pinto and colleagues analyzed the outcome of 24 patients with PAH documented by right heart catheterization, and they report that with a median follow-up of 4 years, 54% died, most of which deaths were attributable to PAH. Patients who receive treatment that reduce their pulmonary pressures have improved survival, suggesting that improvement in monitoring and treatment are critical imperatives for these patients.