title: [Efficacy of oral bosentan for treatment of congenital heart disease-associated pulmonary arterial hypertension].
abstract: OBJECTIVE
  To investigate the effect of oral bosentan in the treatment of congenital heart disease-associated pulmonary arterial hypertension.
  
  
  METHODS
  24 patients with congenital heart disease-associated pulmonary arterial hypertension, including 4 receiving heart surgery and 20 with surgical contraindications, were enrolled in this study. All the patients were given oral bosentan and followed up regularly for analyzing the outcomes and side effects.
  
  
  RESULTS
  One patient was lost to follow up and one patient died. Systolic pulmonary artery pressure showed no significant changes at 2 (93.6 ± 17.2 mmHg) and 4 months (85.7 ± 25.5 mmHg) of bosentan treatment compared to that before the medication (97.8 ± 14.9 mmHg) (P=0.096), but decreased significantly after a 6-month therapy (80.9 ± 25.0 mmHg, P=0.029). The 6-minute walking distance increased significantly after a 2, 4, and 6-month therapy [(488 ± 98.8, 496.3 ± 89.0, and 491.3 ± 114.2 m, respectively; P=0.004, 0.003, and 0.004 vs the distance before medication (317.0 ± 134.1)]. The New York heart functional classification was improved significantly after a 2, 4, and 6-month therapy [(2.0 ± 0.5, 1.8 ± 0.4, and 1.7 ± 0.5, respectively; P<0.001 vs pre-medication score (2.9 ± 0.5)). Hepatic and renal function remained normal, and ALT and AST showed no significant variations during the medication (P>0.05).
  
  
  CONCLUSIONS
  Oral bosentan can effectively relieve the symptoms, decrease pulmonary artery hypertension, and improve exercise tolerance and cardiac function classification in patients with pulmonary artery hypertension associated with congenital heart disease with good safety and mild side effects.
authors.affiliation: Department of Cardiovascular Surgery, PLA General Hospital/Institute of Cardiac Surgery of Chinese PLA, Beijing 100853, China.E-mail: yeweihua71@sina.com.
authors.forename: Weihua
authors.initials: W
authors.lastname: Ye
authors.forename: Bojun
authors.initials: B
authors.lastname: Li
authors.forename: Wei
authors.initials: W
authors.lastname: Sheng
authors.forename: Minghui
authors.initials: M
authors.lastname: Yao
authors.forename: Liang
authors.initials: L
authors.lastname: Shang
authors.forename: Changqing
authors.initials: C
authors.lastname: Gao
mesh_terms: D000284:Administration, Oral; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D006330:Heart Defects, Congenital / Q000150:complications*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology; D013449:Sulfonamides / Q000627:therapeutic use*
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D000077300:Bosentan