title: Long-term trial of bosentan monotherapy for pulmonary arterial hypertension in Japanese patients.
abstract: OBJECTIVE
  To determine the efficacy and safety of long-term bosentan monotherapy in Japanese patients with pulmonary arterial hypertension (PAH).
  
  
  METHODS
  The present study was an extension to a 12-week open-label trial of bosentan in which 21 Japanese patients with PAH received bosentan, 125 mg twice daily. Of the 21 patients in the initial trial, 20 elected to participate in the long-term study and to continue to receive bosentan for up to 3 years.
  
  
  METHODS
  The primary efficacy measure was comparison of World Health Organization (WHO) functional class for pulmonary arterial hypertension following long-term (> 2.5 years) therapy compared with baseline (prior to initiation of bosentan). Secondary outcomes included time from initiation of bosentan therapy to clinical worsening and safety assessments.
  
  
  RESULTS
  Bosentan treatment was continued for a median of 2.7 years (range 0.4-3.0 years); 12 patients received bosentan monotherapy for at least 2.5 years. Following long-term treatment, improvement of WHO functional class compared with baseline was observed in 9/12 patients (75.0%) and in 3/12 patients (25.0%) the functional class remained stable; no patient experienced a worsening of WHO functional class compared with baseline. Overall, long-term treatment with bosentan was well tolerated.
  
  
  CONCLUSIONS
  Long-term treatment with bosentan is well tolerated and is associated with sustained clinical improvement in Japanese patients with PAH. Bosentan, therefore, represents a valuable treatment option for Japanese patients with this devastating disease.
authors.affiliation: Cardiac Bio-Mechanics Center, Doshisha University, Japan. sasayama@wonder.ocn.ne.jp
authors.forename: Shigetake
authors.initials: S
authors.lastname: Sasayama
authors.forename: Toru
authors.initials: T
authors.lastname: Satoh
authors.forename: Tohru
authors.initials: T
authors.lastname: Izumi
authors.forename: Shunji
authors.initials: S
authors.lastname: Yoshida
authors.forename: Shingo
authors.initials: S
authors.lastname: Kyotani
authors.forename: Nobuhiro
authors.initials: N
authors.lastname: Tahara
mesh_terms: D000328:Adult; D047228:Angiotensin II Type 1 Receptor Blockers / Q000627:therapeutic use*; D000077300:Bosentan; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy*; D007564:Japan; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D012720:Severity of Illness Index; D013449:Sulfonamides / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D047228:Angiotensin II Type 1 Receptor Blockers; D013449:Sulfonamides; D000077300:Bosentan