title: Long-term safety, tolerability and efficacy of bosentan in adults with pulmonary arterial hypertension associated with congenital heart disease.
abstract: OBJECTIVE
  To examine long-term safety and efficacy of bosentan--an oral dual endothelin receptor antagonist--in patients with pulmonary hypertension associated with congenital heart disease or Eisenmenger's syndrome.
  
  
  METHODS
  Retrospective study.
  
  
  METHODS
  Tertiary cardiology referral centre.
  
  
  METHODS
  All adult patients with pulmonary arterial hypertension associated with congenital heart disease treated with bosentan at the Royal Brompton Adult Congenital Heart Centre were included.
  
  
  METHODS
  Oxygen saturation, functional (WHO) class, 6-minute walk test distance and liver enzymes were analysed.
  
  
  RESULTS
  Eighteen patients (14 female) with pulmonary arterial hypertension associated with congenital heart disease (15 patients with Eisenmenger's syndrome) with a mean (SD) age of 41 (9) years (range 23-69) were included. Median follow-up was 29 months (range 1-39). One patient died during follow-up. Patients tolerated bosentan well and no significant rise in liver transaminases was seen. Arterial oxygen saturation remained stable throughout follow-up. Mean (SD) functional class (p = 0.001) and the 6-minute walk test distance improved compared with baseline (284 (144) vs 363 (124) m, 380 (91) m and 408 (114) m at baseline, 0-6 months, 6-12 months and 1-2 years of treatment, respectively; p<0.05 for each).
  
  
  CONCLUSIONS
  Bosentan appears to be safe and well tolerated in adults with pulmonary arterial hypertension associated with congenital heart disease or Eisenmenger's syndrome during mid- to long-term follow-up. In addition, functional class and the 6-minute walk test distance improved and this effect was maintained for up to 2 years of bosentan treatment.
authors.affiliation: Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, and the National Heart and Lung Institute, Imperial College of Science and Medicine, London, UK.
authors.forename: Gerhard-Paul
authors.initials: GP
authors.lastname: Diller
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Dimopoulos
authors.forename: Mehmet G
authors.initials: MG
authors.lastname: Kaya
authors.forename: Carl
authors.initials: C
authors.lastname: Harries
authors.forename: Anselm
authors.initials: A
authors.lastname: Uebing
authors.forename: Wei
authors.initials: W
authors.lastname: Li
authors.forename: Evdokia
authors.initials: E
authors.lastname: Koltsida
authors.forename: J Simon R
authors.initials: JS
authors.lastname: Gibbs
authors.forename: Michael A
authors.initials: MA
authors.lastname: Gatzoulis
mesh_terms: D000328:Adult; D000368:Aged; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D004541:Eisenmenger Complex / Q000150:complications / Q000188:drug therapy / Q000503:physiopathology; D017079:Exercise Tolerance; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D008875:Middle Aged; D010100:Oxygen / Q000097:blood; D012449:Safety; D013449:Sulfonamides / Q000627:therapeutic use*
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D000077300:Bosentan; D010100:Oxygen