title: The Bosentan Patient Registry: long-term survival in pulmonary arterial hypertension.
abstract: OBJECTIVE
  The Bosentan Patient Registry (BPR) was a prospective, multicentre, Australian registry funded by Actelion Pharmaceuticals. The primary aim of the registry was to collect survival data in patients with pulmonary arterial hypertension (PAH) treated with bosentan.
  
  
  METHODS
  The BPR was initiated in 15 specialized PAH centres. All patients on or starting bosentan were invited to enrol. Treating physicians notified the registry if patients discontinued bosentan, because of either a change in therapy, transplantation, intervention or death. Survival data were validated against the Australian Institute of Health and Welfare National Death Index.
  
  
  RESULTS
  Between 2004 and 2007, a total of 528 patients (mean age 59 ± 17 years) were enrolled representing 69% of patients either previously taking or initiated on bosentan during that time. The BPR population was generally older with more advanced functional deficit than patients enrolled in randomized, placebo-controlled trials. Aetiology was idiopathic (iPAH) in 58% and connective tissue disease related (scleroderma (SSc)-PAH) in 42%. For iPAH patients, World Health Organisation functional classes II, III and IV at enrolment was 8.2%, 66.4% and 20.5%, and for the SSc-PAH cohort, 3.2%, 75.8% and 17.9% respectively. The observed annual mortality was 11.8% in patients with iPAH and 16.6% in patients SSc-PAH.
  
  
  CONCLUSIONS
  This large Australian registry provides 'real life' information on the characteristics and management of PAH in clinical practice. Treatment with bosentan improved survival outcomes in both iPAH and SSc-PAH compared with historical controls. Age, disease severity and aetiology were critical factors in determining clinical outcomes.
authors.affiliation: St Vincent's Hospital, Sydney, John Wlodarczyk Consulting Services, Newcastle, New South Wales, Australia. amkeogh@stvincents.com.au
authors.forename: A
authors.initials: A
authors.lastname: Keogh
authors.forename: G
authors.initials: G
authors.lastname: Strange
authors.forename: K
authors.initials: K
authors.lastname: McNeil
authors.forename: T J
authors.initials: TJ
authors.lastname: Williams
authors.forename: E
authors.initials: E
authors.lastname: Gabbay
authors.forename: S
authors.initials: S
authors.lastname: Proudman
authors.forename: R G
authors.initials: RG
authors.lastname: Weintraub
authors.forename: J
authors.initials: J
authors.lastname: Wlodarczyk
authors.forename: B
authors.initials: B
authors.lastname: Dalton
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D001315:Australia / Q000453:epidemiology; D000077300:Bosentan; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012042:Registries; D013449:Sulfonamides / Q000627:therapeutic use*; D015996:Survival Rate / Q000639:trends; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D000077300:Bosentan