title: The effect of bosentan in patients with a failing Fontan circulation.
abstract: OBJECTIVE
  To investigate the effect of bosentan in patients with a failing Fontan circulation.
  
  
  METHODS
  A multicentric open label, non-controlled study.
  
  
  METHODS
  5 tertiary care centres for congenital cardiology.
  
  
  METHODS
  We included 10 patients with a failing Fontan circulation. Their median age at inclusion was 12.12 years, with a range from 4.41 to 33,41 years. The median interval between the Fontan operation and inclusion was 7.84 years, with a range from 1.96 to 12,18 years. Participants received half the usual dose of bosentan for 4 weeks, and then the full dose for a further 12 weeks. MAIN MEASURES OF OUTCOMES: We assessed saturations of oxygen at rest and during exercise, using a 6 minutes walk test, at baseline, and during and after 16 weeks of treatment. At each visit, we assessed blood chemistry and hepatic function, and asked the patients to complete a questionnaire concerning quality of life. All medical events and possible side effects were recorded.
  
  
  RESULTS
  Of the cohort, 1 patient withdrew. The changes in saturations of oxygen, exercise performance, and scores for the questionnaire did not reach statistical significance for the whole group. We noted, nonetheless, that saturations of oxygen and/or exercise capacity improved in 5 of the patients. This was further confirmed when those patients deteriorated again when the drug was discontinued.
  
  
  CONCLUSIONS
  Our study failed to show significant improvement after 3 months of treatment with bosentan in a small group of patients with failing Fontan circulations. Some individuals, nonetheless, did improve. When planning larger trials, it would be better to identify those patients who might potentially benefit from the treatment prior to commencing the trial.
authors.affiliation: Department of Pediatric Cardiology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium.
authors.forename: Caroline
authors.initials: C
authors.lastname: Ovaert
authors.forename: Daisy
authors.initials: D
authors.lastname: Thijs
authors.forename: Daniel
authors.initials: D
authors.lastname: Dewolf
authors.forename: Jaap
authors.initials: J
authors.lastname: Ottenkamp
authors.forename: Hugues
authors.initials: H
authors.lastname: Dessy
authors.forename: Philip
authors.initials: P
authors.lastname: Moons
authors.forename: Marc
authors.initials: M
authors.lastname: Gewillig
authors.forename: Luc
authors.initials: L
authors.lastname: Mertens
mesh_terms: D000293:Adolescent; D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000009:adverse effects*; D006330:Heart Defects, Congenital / Q000503:physiopathology / Q000628:therapy*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D008297:Male; D010865:Pilot Projects; D011652:Pulmonary Circulation / Q000187:drug effects / Q000502:physiology*; D012189:Retrospective Studies; D013449:Sulfonamides / Q000627:therapeutic use*; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D000077300:Bosentan