title: Long-term bosentan in chronic thromboembolic pulmonary hypertension.
abstract: BACKGROUND
  There is no approved pharmacological treatment for patients with chronic thromboembolic pulmonary hypertension (CTEPH) who are not suitable for pulmonary endarterectomy (PEA).
  
  
  OBJECTIVE
  The study investigates the effect of the dual endothelin receptor antagonist bosentan on exercise tolerance (6-min walking distance, 6MWD) and right ventricular function (Tei index) in patients with CTEPH over 24 months.
  
  
  METHODS
  Twelve consecutive patients (5 males and 7 females) with CTEPH not eligible for PEA or following partial or complete failure of PEA were included in a non-randomized, open-label prospective study. All patients were WHO class III. They were included, if progressive pulmonary hypertension was diagnosed despite best supportive treatment. Bosentan was started at 62.5 mg b.i.d. and increased to the final dose of 125 mg b.i.d.
  
  
  RESULTS
  6MWD and the Tei index were assessed every 6 months. We observed a significant increase in 6MWD from 319 +/- 85.0 m at baseline to 391 +/- 76.9 m at 6 months and a significant decrease in the Tei index from 0.39 +/- 0.10 at baseline to 0.34 +/- 0.08 at 6 months. This improvement was maintained over 24 months (6 MWD: 381 +/- 101 m; Tei index: 0.31 +/- 0.03). Six patients exhibited an improvement in WHO class at 6, 12 and 18 months, 5 demonstrated improvement at the 24-month follow-up. The remainder were stable throughout the study period.
  
  
  CONCLUSIONS
  This is the first study demonstrating a long-term beneficial effect of bosentan on exercise tolerance (6MWD) and right heart function (Tei index) in CTEPH.
authors.affiliation: Department of Respiratory Medicine, University of Leipzig, Leipzig, Germany. seyfarth.leipzig@freenet.de
authors.forename: Hans-Jurgen
authors.initials: HJ
authors.lastname: Seyfarth
authors.forename: Stefan
authors.initials: S
authors.lastname: Hammerschmidt
authors.forename: Hans
authors.initials: H
authors.lastname: Pankau
authors.forename: Jorg
authors.initials: J
authors.lastname: Winkler
authors.forename: Hubert
authors.initials: H
authors.lastname: Wirtz
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D002908:Chronic Disease; D017079:Exercise Tolerance / Q000187:drug effects; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy*; D053208:Kaplan-Meier Estimate; D008297:Male; D011446:Prospective Studies; D013449:Sulfonamides / Q000627:therapeutic use*; D013923:Thromboembolism; D016896:Treatment Outcome; D016278:Ventricular Function, Right / Q000187:drug effects / Q000502:physiology
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D000077300:Bosentan