title: [Outcome of oral bosentan in children with congenital heart disease associated pulmonary arterial hypertension].
abstract: OBJECTIVE
  To investigate the outcome of dual endothelin receptor antagonist bosentan in children with congenital heart disease (CHD) associated pulmonary arterial hypertension (PAH).
  
  
  METHODS
  A total of 32 children were recruited into this prospective and observational study. Among them, there were 18 cases with left-to-right shunt and 14 cases with elevated pulmonary vascular resistance (PVR) in functional single ventricle (FSV). All the cases were treated with oral bosentan, initiated from 90 days before or 8 years after operation, and were followed up periodically to analyze the clinical outcome and monitor its side effects.
  
  
  RESULTS
  In the left-to-right shunt group, pulmonary arterial pressure (PAP) was measured at (57 +/- 26), (52 +/- 31) and (46 +/- 22) mm Hg after oral bosentan therapy at 1, 2 and 3 months respectively. The measurements significantly decreased as compared with the pre-dosing level of (74 +/- 15) mm Hg (P < 0.05). After a 3-months therapy of bosentan, World Health Organization functional class (WHO FC) improved significantly (P < 0.01). In the elder cases, the 6-minute walking distance after a 3-month bosentan therapy significantly increased as compared with the pre-dosing level, i. e. (497 +/- 56) vs (424 +/- 31) m (P < 0.05). In the FSV group, as compared with the pre-dosing level, the transcutaneous oxygen saturation increased significantly in the last follow-up during bosentan exposure, i. e. (86 +/- 5)% vs (78 +/- 6)% (P < 0.01). WHO FC improved significantly (P < 0.01) and the incidence of facial edema and pleural effusion was significant lower (P < 0.05) in the last follow-up for the treatment group. Patients tolerated bosentan well and no significant rise in hepatic transaminases was observed.
  
  
  CONCLUSIONS
  Bosentan is safe in treating CHD associated PAH in children. In left-to-right shunt cases, oral bosentan can reduce PAP and improve both WHO FC and exercise capacity. And it can also improve WHO FC and transcutaneous oxygen saturation in FSV and reduces the occurrence of elevated PVR-related complications.
authors.affiliation: Department of Cardiothoracic Vascular Surgery, Shanghai Children's Medical Center, Medical College of Shanghai Jiaotong University, Shanghai 200127, China.
authors.forename: Zhuo-ming
authors.initials: ZM
authors.lastname: Xu
authors.forename: Li-Min
authors.initials: LM
authors.lastname: Zhu
authors.forename: Xiao-Man
authors.initials: XM
authors.lastname: Cai
authors.forename: Gang
authors.initials: G
authors.lastname: Ji
authors.forename: Jin-Fen
authors.initials: JF
authors.lastname: Liu
authors.forename: Zhao-Kang
authors.initials: ZK
authors.lastname: Su
mesh_terms: D000293:Adolescent; D000959:Antihypertensive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D000077300:Bosentan; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology*; D007223:Infant; D008297:Male; D011446:Prospective Studies; D013449:Sulfonamides / Q000009:adverse effects / Q000627:therapeutic use*
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D000077300:Bosentan