title: Bosentan therapy for inoperable chronic thromboembolic pulmonary hypertension.
abstract: BACKGROUND
  Bosentan, an oral endothelin (ET)-A/ET-B receptor antagonist, is effective in the treatment of pulmonary arterial hypertension.
  
  
  OBJECTIVE
  To investigate the safety and efficacy of bosentan therapy in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH).
  
  
  METHODS
  Case series.
  
  
  METHODS
  Pulmonary Hypertension Unit of the Medical University of Vienna, Austria.
  
  
  METHODS
  Sixteen patients (9 women and 7 men; mean age +/- SD, 70 +/- 13 years).
  
  
  METHODS
  Off-label bosentan treatment over 6 months.
  
  
  METHODS
  Changes from baseline in liver enzymes, New York Heart Association (NYHA) functional class, 6-min walking distance (6-MWD), and serum amino-terminal pro-brain natriuretic peptide (proBNP).
  
  
  RESULTS
  After 6 months, NYHA functional class improved by one class in 11 patients. Mean 6-MWDs increased from 299 +/- 131 m at baseline to 391 +/- 110 m at 6 months (p = 0.01). In parallel, proBNP decreased from 3,365 +/- 2,923 to 1,755 +/- 1,812 pg/mL (p = 0.01). Neither aspartate aminotransferase (25 +/- 2 U/L vs 25 +/- 2 U/L, p = 0.25) nor alanine aminotransferase (23 +/- 12 U/L vs 24 +/- 9 U/L, p = 0.57) changed significantly. Limitations of the study were uncontrolled design and small sample size.
  
  
  CONCLUSIONS
  Our study suggests a beneficial effect of the oral dual ET receptor antagonist bosentan in patients with inoperable CTEPH, urging the need for a randomized, placebo-controlled trial.
authors.affiliation: Department of Cardiology, Medical University of Vienna, Währinger Gürtel 18-20, 1090 Vienna, Austria.
authors.forename: Diana
authors.initials: D
authors.lastname: Bonderman
authors.forename: Regina
authors.initials: R
authors.lastname: Nowotny
authors.forename: Nika
authors.initials: N
authors.lastname: Skoro-Sajer
authors.forename: Johannes
authors.initials: J
authors.lastname: Jakowitsch
authors.forename: Christopher
authors.initials: C
authors.lastname: Adlbrecht
authors.forename: Walter
authors.initials: W
authors.lastname: Klepetko
authors.forename: Irene M
authors.initials: IM
authors.lastname: Lang
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000410:Alanine Transaminase / Q000097:blood; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D001219:Aspartate Aminotransferases / Q000097:blood; D000077300:Bosentan; D065128:Endothelin Receptor Antagonists; D005080:Exercise Test; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy*; D008111:Liver Function Tests; D008297:Male; D008875:Middle Aged; D013449:Sulfonamides / Q000627:therapeutic use*; D013923:Thromboembolism / Q000188:drug therapy*; D016138:Walking
chemical_list: D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D013449:Sulfonamides; D001219:Aspartate Aminotransferases; D000410:Alanine Transaminase; D000077300:Bosentan