title: Long-term outcomes in pulmonary arterial hypertension in the first-line epoprostenol or first-line bosentan era.
abstract: BACKGROUND
  The aim of this study was to describe the long-term outcomes in idiopathic pulmonary arterial hypertension (IPAH) treated with first-line bosentan or intravenous (IV) epoprostenol, and additional therapy as needed.
  
  
  METHODS
  In a single-center, retrospective, longitudinal cohort, data on right heart catheterization, 6-minute walk distance (6MWD), disease progression and mortality were collected. Outcomes were assessed in first-line bosentan and first-line epoprostenol patients. To reduce selection bias due to differences between groups, two independent analyses were performed. First, a comparison was made of World Health Organization (WHO) Functional Class (FC) III patients. Second, to control for disease severity, a matched-pairs analysis was performed, with matching according to baseline cardiac output and exercise capacity and irrespective of FC at baseline.
  
  
  RESULTS
  Thirty-seven IPAH patients initiated first-line bosentan treatment and 37 first-line IV epoprostenol. Twenty-nine of the bosentan patients and 16 of the IV epoprostenol patients were in WHO FC III; demographic profiles were similar, although hemodynamic measurements and 6MWD suggested more severe disease in the IV epoprostenol group at treatment initiation. At 1 and 3 years, median change in 6MWD for patients initiating bosentan was +54 m (95% confidence interval: -3 to 76) and +71 m (-123 to 116), respectively, and +92 m (17 to 128) and +142 m (-6 to 242) for those on IV epoprostenol. Absence of disease progression of WHO FC III at 1 and 3 years was 72% and 45% with bosentan and 75% and 44% with IV epoprostenol, respectively. Survival at 1 and 3 years was 93% and 89% with bosentan and 94% and 75% with IV epoprostenol, respectively. Results were confirmed in matched-pairs analysis of 16 bosentan and 16 IV epoprostenol patients with similar disease severity.
  
  
  CONCLUSIONS
  First-line epoprostenol treatment may lead to greater improvement in exercise capacity than first-line bosentan. However, these greater exercise improvements did not translate into longer time to disease progression or survival.
authors.affiliation: Department of Pulmonology, VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: Wouter
authors.initials: W
authors.lastname: Jacobs
authors.forename: Anco
authors.initials: A
authors.lastname: Boonstra
authors.forename: Monika
authors.initials: M
authors.lastname: Brand
authors.forename: Daniel M
authors.initials: DM
authors.lastname: Rosenberg
authors.forename: Berthold
authors.initials: B
authors.lastname: Schaaf
authors.forename: Pieter E
authors.initials: PE
authors.lastname: Postmus
authors.forename: Anton
authors.initials: A
authors.lastname: Vonk Noordegraaf
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D011464:Epoprostenol / Q000627:therapeutic use*; D017079:Exercise Tolerance; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013449:Sulfonamides / Q000627:therapeutic use*; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D011464:Epoprostenol; D000077300:Bosentan