Survival in Primary Pulmonary Hypertension

Abstract

Background— Primary pulmonary hypertension (PPH) is a severe and progressive disease. Without treatment, the median survival is 2.8 years, with survival rates of 68%, 48%, and 34% at 1, 3, and 5 years, respectively. Intravenous epoprostenol was the first Food and Drug Administration–approved therapy for PPH. The long-term impact that epoprostenol has made on PPH remains to be defined.