title: Long-term outcome and effects of oral bosentan therapy in Taiwanese patients with advanced idiopathic pulmonary arterial hypertension.
abstract: METHODS
  We report on the long-term outcome and effects of bosentan treatment in Taiwanese patients with advanced (functional class III or IV) idiopathic pulmonary arterial hypertension (IPAH).
  
  
  METHODS
  IPAH patients on stable bosentan therapy for more than 12 months and regularly monitored were eligible for this prospective uncontrolled study. Patients were evaluated for several clinical parameters, both measured at the time of initiation of bosentan therapy and after 12 months on therapy: New York Heart Association functional class (NYHA FC), change in 6-min walk distance (6MWD), right ventricle ejection fraction (RVEF), cardiothoracic ratio (CTR), and pulmonary functional status.
  
  
  RESULTS
  Twelve of 15 patients met eligibility requirements and were enrolled. Their mean age was 37.6+/-12.9 years and 92% were female. Six (50%) patients were in NYHA FC IV and the others were in NYHA FC III at baseline. Three (25%) patients were chronic hepatitis C virus (HCV) carriers, with normal liver function. After 12 months of bosentan treatment, 6-MWD, RVEF, and pulmonary function all increased significantly. CTR and NYHA FC both decreased significantly. Oral bosentan was well tolerated and there was no episode of liver dysfunction that required adjustment of the bosentan dosage or discontinuance of therapy.
  
  
  CONCLUSIONS
  Long-term treatment with oral bosentan appears to have beneficial effects on functional status, exercise capacity, right heart function, and pulmonary function in Taiwanese patients with advanced IPAH, regardless of whether or not they presented with chronic HCV infection.
authors.affiliation: Department of Surgery, National Taiwan University Hospital, National Taiwan University College of Medicine, No. 7, Chung-Shan South Road, Taipei, Taiwan.
authors.forename: Hsao-Hsun
authors.initials: HH
authors.lastname: Hsu
authors.forename: Jin-Shing
authors.initials: JS
authors.lastname: Chen
authors.forename: Robert J
authors.initials: RJ
authors.lastname: Chen
authors.forename: Wen-Je
authors.initials: WJ
authors.lastname: Ko
authors.forename: Shuenn-Wen
authors.initials: SW
authors.lastname: Kuo
authors.forename: En-Ting
authors.initials: ET
authors.lastname: Wu
authors.forename: Mei-Hwan
authors.initials: MH
authors.lastname: Wu
authors.forename: Jou-Kou
authors.initials: JK
authors.lastname: Wang
authors.forename: Yung-Chie
authors.initials: YC
authors.lastname: Lee
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D000077300:Bosentan; D005080:Exercise Test; D005260:Female; D005541:Forced Expiratory Volume / Q000187:drug effects; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D013318:Stroke Volume / Q000187:drug effects; D013449:Sulfonamides / Q000009:adverse effects / Q000627:therapeutic use*; D016896:Treatment Outcome; D014797:Vital Capacity / Q000187:drug effects
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D000077300:Bosentan