title: Bosentan therapy for inoperable chronic thromboembolic pulmonary hypertension.
abstract: OBJECTIVE
  We performed an open-label multicenter study to evaluate the safety and efficacy of the dual endothelin receptor antagonist bosentan in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH).
  
  
  METHODS
  Nineteen patients with inoperable CTEPH were enrolled.
  
  
  METHODS
  The primary end point was a change in pulmonary vascular resistance (PVR). Secondary end points included 6-min walk test, peak oxygen uptake (V(O2)), New York Heart Association functional class, serum levels of N-terminal-pro brain natriuretic peptide (NT-pro-BNP), and various other hemodynamic parameters.
  
  
  RESULTS
  After 3 months of treatment with bosentan, PVR decreased from 914 +/- 329 to 611 +/- 220 dyne.s.cm(-5) (p < 0.001). Functional class and peak V(O2) remained unchanged, but 6-min walk distance increased from 340 +/- 102 to 413 +/- 130 m (p = 0.009), and serum NT-pro BNP levels improved from 2,895 +/- 2,620 to 2,179 +/- 2,301 (p = 0.027). One patient died, presumably from influenza A infection, and another patient experienced progressive fluid retention despite reduction of PVR. Other than that, treatment was well tolerated by all patients.
  
  
  CONCLUSIONS
  This open-label pilot trial suggests that bosentan may offer a therapeutic option for patients with inoperable CTEPH. Randomized controlled trials are warranted to confirm these findings.
authors.affiliation: Department of Respiratory Medicine, Hannover Medical School, 30623 Hannover, Germany. hoeper.marius@mh-hannover.de
authors.forename: Marius M
authors.initials: MM
authors.lastname: Hoeper
authors.forename: Thorsten
authors.initials: T
authors.lastname: Kramm
authors.forename: Heinrike
authors.initials: H
authors.lastname: Wilkens
authors.forename: Christine
authors.initials: C
authors.lastname: Schulze
authors.forename: Hans Joachim
authors.initials: HJ
authors.lastname: Schäfers
authors.forename: Tobias
authors.initials: T
authors.lastname: Welte
authors.forename: Eckhard
authors.initials: E
authors.lastname: Mayer
mesh_terms: D000368:Aged; D000925:Anticoagulants / Q000627:therapeutic use; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D004232:Diuretics / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D020097:Natriuretic Peptide, Brain / Q000097:blood; D010101:Oxygen Consumption; D010865:Pilot Projects; D013449:Sulfonamides / Q000627:therapeutic use*; D013923:Thromboembolism / Q000503:physiopathology*; D014655:Vascular Resistance; D016138:Walking / Q000502:physiology
chemical_list: D000925:Anticoagulants; D000959:Antihypertensive Agents; D004232:Diuretics; D013449:Sulfonamides; D020097:Natriuretic Peptide, Brain; D000077300:Bosentan