title: Clinical efficacy and survival with first-line inhaled iloprost therapy in patients with idiopathic pulmonary arterial hypertension.
abstract: OBJECTIVE
  To describe the long-term clinical efficacy of inhaled iloprost as first-line vasodilator mono-therapy in patients with idiopathic pulmonary arterial hypertension (IPAH).
  
  
  RESULTS
  Seventy-six IPAH patients were prospectively identified and treated with inhaled iloprost. Clinical, haemodynamic, and exercise parameters were obtained at baseline, after 3 and 12 months of therapy and yearly thereafter. Four endpoints were prospectively defined as follows: (i) death, (ii) transplantation, (iii) switch to intravenous (i.v.) therapy, or (iv) addition of or switch to other active oral therapy. During follow-up (535+/-61 days), 11 patients died, six were transplanted, 25 were switched to i.v. prostanoids, 16 received additional or other oral therapy, and 12 patients discontinued iloprost inhalation for other reasons. Event-free survival at 3, 12, 24, 36, 48, and 60 months was 81, 53, 29, 20, 17 and 13%, respectively. Among haemodynamic and exercise parameters, mixed venous oxygen saturation (P<0.001), right atrial pressure (P<0.001), and peak oxygen uptake (P=0.002) were associated with event-free survival.
  
  
  CONCLUSIONS
  In this study, only a minority of patients could be stabilized with inhaled iloprost mono-therapy during a follow-up period of up to 5 years. In the presence of multiple treatment options, chronic iloprost inhalation as mono-therapy appears to have a limited role.
authors.affiliation: Department of Cardiology, DRK-Kliniken Berlin Westend, Spandauer Damm 130, 14050 Berlin, Germany. c.opitz@drk-kliniken-westend.de
authors.forename: Christian F
authors.initials: CF
authors.lastname: Opitz
authors.forename: Roland
authors.initials: R
authors.lastname: Wensel
authors.forename: Jörg
authors.initials: J
authors.lastname: Winkler
authors.forename: Michael
authors.initials: M
authors.lastname: Halank
authors.forename: Leonhard
authors.initials: L
authors.lastname: Bruch
authors.forename: Franz-Xaver
authors.initials: FX
authors.lastname: Kleber
authors.forename: Gert
authors.initials: G
authors.lastname: Höffken
authors.forename: Stefan D
authors.initials: SD
authors.lastname: Anker
authors.forename: Abdissa
authors.initials: A
authors.lastname: Negassa
authors.forename: Stephan B
authors.initials: SB
authors.lastname: Felix
authors.forename: Roland
authors.initials: R
authors.lastname: Hetzer
authors.forename: Ralf
authors.initials: R
authors.lastname: Ewert
mesh_terms: D000280:Administration, Inhalation; D000328:Adult; D005080:Exercise Test; D005260:Female; D005500:Follow-Up Studies; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000401:mortality / Q000503:physiopathology; D016285:Iloprost / Q000008:administration & dosage*; D008297:Male; D016016:Proportional Hazards Models; D011446:Prospective Studies; D016019:Survival Analysis; D014665:Vasodilator Agents / Q000008:administration & dosage*
chemical_list: D014665:Vasodilator Agents; D016285:Iloprost