title: Safety and tolerability evaluation of oral bosentan in adult congenital heart disease associated pulmonary arterial hypertension: a systematic review and meta-analysis.
abstract: OBJECTIVE
  In this study, we performed a systematic review and meta-analysis of oral bosentan in adult congenital heart disease associated pulmonary arterial hypertension (CHD-PAH) to evaluate its safety and tolerability.
  
  
  METHODS
  Online electronic database including PubMed, EMBASE and Springer were searched from October 2006 to October 2013 to collect the clinical studies or cohort trials on CHD-PAH with bosentan treatment. Weight Mean Difference (WMD) and Standard Mean Difference (SMD) were used to evaluate the treatment safety and tolerability. Review Manager (RevMan) version 5.0 was performed for the data analysis.
  
  
  RESULTS
  Totally 8 studies including 215 patients with CHD-PAH were enrolled in this research. With a period of 3-6 months oral bosentan treatment in patients, there were no significant differences in the scores of resting oxygen saturation (Resting SpO2), post-6-MWT SpO2 after 6-minutes' walktest (6-MWT) and Borg dyspnea index score (BDIs) compared with the baseline; the walking distance on 6-MWT increased significantly. With a period of one year or more oral bosentan treatment, the scores of resting SpO2 and post-6-MWT SpO2 increased significantly; there was no significant difference in BDIs and walking distance on 6-MWT.
  
  
  CONCLUSIONS
  The short-term treatment with oral bosentan could increase walking distance on 6-MWT, and long-term treatment could increase the Resting SpO2 in CHD-PAH patients. Oral bosentan in CHD-PAH patients was safe and well tolerated.
authors.affiliation: Department of Respiratory Medicine, Sichuan Academy of Medical Science, People's Hospital of Sichuan Province, Chengdu, China. guoluanti@hotmail.com  
authors.forename: L
authors.initials: L
authors.lastname: Guo
authors.forename: Y-J
authors.initials: YJ
authors.lastname: Liu
authors.forename: Z-L
authors.initials: ZL
authors.lastname: Xie
mesh_terms: D000284:Administration, Oral; D000328:Adult; D000959:Antihypertensive Agents / Q000008:administration & dosage* / Q000009:adverse effects; D000077300:Bosentan; D004341:Drug Evaluation / Q000379:methods; D005260:Female; D006330:Heart Defects, Congenital / Q000175:diagnosis* / Q000188:drug therapy* / Q000453:epidemiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000188:drug therapy* / Q000453:epidemiology; D008297:Male; D013449:Sulfonamides / Q000008:administration & dosage* / Q000009:adverse effects
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D000077300:Bosentan