title: A retrospective study of bosentan in pulmonary arterial hypertension associated with congenital heart disease.
abstract: BACKGROUND
  Pulmonary Arterial Hypertension (PAH) plays a significant role in morbidity and mortality of patients with congenital heart disease (CHD). Bosentan, a dual endothelin receptor antagonist has been approved for PAH patients with Eisenmenger physiology (EP). The authors retrospectively reviewed the efficacy and safety of bosentan in Thai PAH patients associated with CHD.
  
  
  METHODS
  The study population was obtained from the databases of the CHD patients at Siriraj Hospital from October 2004 to April 2007 who received 6 months of bosentan treatment. Inclusion criteria are: CHD with Eisenmenger physiology (EP) or those with severe PAH after surgical repair or interventional cardiac catheterization. Clinical characteristics including the 6-- minute walk test (6MWT) distances, oxygen saturation (O2 sat), New York Heart Association (NYHA) functional class, and right ventricular systolic pressure (RVSP) at baseline were compared with those at 1, 3, and 6 months post bosentan treatment. Signs and symptoms of adverse events were also recorded.
  
  
  RESULTS
  There were 11 patients from among those who fitted the inclusion criteria and whose records were examined. Their average age was 51.1 +/- 10.1 years old (13-61 years old). Patients were divided into 2 groups; Group A (6 patients) was PAH with EP and Group B (5 patients) was PAH post intervention. In group A, the 6MWT increased from 151 +/- 69 meters to 293 +/- 61 meters (p = 0.001) with the average increase of 38 +/- 61 meters. The 2O sat increased from 83 +/- 12.7% to 91.8 +/- 5.6% (p = 0.038) with an average increase of 1.4 +/- 0.07%. There was no significant change in right ventricular systolic pressure (RVSP). In group B, there was a trend in 6MWT improvement from 274 +/- 69 meters to 312 +/- 38 meters but this was not statistically different. There were improvements in the NYHA functional class in both groups. There was no significant increase in serum aminotransferase at the end of 6 months in each patient.
  
  
  CONCLUSIONS
  There are benefits of bosentan for treatment of severe PAH in CHD, especially in patients with Eisenmenger physiology. Obvious benefits are an improvement of 6MWT and O2 sat.
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand. sikdr@mahidol.ac.th
authors.forename: Kritvikrom
authors.initials: K
authors.lastname: Durongpisitkul
authors.forename: Decho
authors.initials: D
authors.lastname: Jakrapanichakul
authors.forename: Suree
authors.initials: S
authors.lastname: Sompradikul
mesh_terms: D000293:Adolescent; D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D019992:Databases as Topic; D004541:Eisenmenger Complex; D016232:Endothelins / Q000187:drug effects; D005080:Exercise Test; D005260:Female; D006304:Health Status; D006305:Health Status Indicators; D006330:Heart Defects, Congenital / Q000150:complications*; D006352:Heart Ventricles; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology; D008297:Male; D008875:Middle Aged; D011651:Pulmonary Artery / Q000187:drug effects / Q000473:pathology*; D012189:Retrospective Studies; D013449:Sulfonamides / Q000627:therapeutic use*; D013599:Systole; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents; D016232:Endothelins; D013449:Sulfonamides; D000077300:Bosentan