title: Impact of sildenafil therapy on pulmonary arterial hypertension in adults with congenital heart disease.
abstract: BACKGROUND
  It has been demonstrated that sildenafil is effective in patients with pulmonary arterial hypertension (PAH). However, the impact of sildenafil on PAH in adults with congenital heart disease (CHD) has been less investigated.
  
  
  OBJECTIVE
  In this prospective, open-label, uncontrolled and multicenter study, 60 patients with PAH related to CHD received oral sildenafil (75 mg/day) for 12 weeks. The enrolled patients underwent six-minute walk test (SMWT) and cardiac catheterization at the beginning and the end of the 12 weeks. The primary end point was the changes in exercise capacity assessed by SMWT; the secondary end point included assessment of functional class, evaluation of cardiopulmonary hemodynamics, and clinical worsening (defined as death, transplantation, and rehospitalization for PAH). Drug safety and tolerability were also examined.
  
  
  RESULTS
  Oral sidenafil significantly increased SMWT distances (422.94 ± 76.95 m vs. 371.99 ± 78.73 m, P < 0.0001). There was also remarkable improvement in Borg dyspnea score (2.1 ± 1.32 vs. 2.57 ± 1.42, P = 0.0307). Moreover, significant improvements in World Healthy Organization (WHO) functional class and cardiopulmonary hemodynamics were also discovered (mean pulmonary artery pressure, P = 0.0002; cardiac index, P < 0.0001; pulmonary vascular resistance, P < 0.0001). Side effects in this study were mild and consistent with reported studies. None of the enrolled patients experienced significant clinical worsening.
  
  
  CONCLUSIONS
  This study confirmed and extended previous studies. It suggested that oral sildenafil was safe and effective for the treatment of adult patients with CHD-related PAH.
authors.affiliation: Center for Diagnosis and Management of Pulmonary Vascular Diseases, Department of Cardiology, Cardiovascular Institute and Fu Wai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
authors.forename: Xian-Ling
authors.initials: XL
authors.lastname: Lu
authors.forename: Chang-Ming
authors.initials: CM
authors.lastname: Xiong
authors.forename: Guang-Liang
authors.initials: GL
authors.lastname: Shan
authors.forename: Xian-Yang
authors.initials: XY
authors.lastname: Zhu
authors.forename: Bing-Xiang
authors.initials: BX
authors.lastname: Wu
authors.forename: Guang-Hua
authors.initials: GH
authors.lastname: Wu
authors.forename: Zhi-Hong
authors.initials: ZH
authors.lastname: Liu
authors.forename: Xin-Hai
authors.initials: XH
authors.lastname: Ni
authors.forename: Xian-Sheng
authors.initials: XS
authors.lastname: Cheng
authors.forename: Qing
authors.initials: Q
authors.lastname: Gu
authors.forename: Zhi-Hu
authors.initials: ZH
authors.lastname: Zhao
authors.forename: Duan-Zhen
authors.initials: DZ
authors.lastname: Zhang
authors.forename: Wei-Min
authors.initials: WM
authors.lastname: Li
authors.forename: Cheng
authors.initials: C
authors.lastname: Zhang
authors.forename: Hong-Yan
authors.initials: HY
authors.lastname: Tian
authors.forename: Ya-Juan
authors.initials: YJ
authors.lastname: Guo
authors.forename: Tao
authors.initials: T
authors.lastname: Guo
authors.forename: Hong-Min
authors.initials: HM
authors.lastname: Liu
authors.forename: Wei-Jun
authors.initials: WJ
authors.lastname: Zhang
authors.forename: Hong
authors.initials: H
authors.lastname: Gu
authors.forename: Shi-An
authors.initials: SA
authors.lastname: Huang
authors.forename: Jian-Ying
authors.initials: JY
authors.lastname: Chen
authors.forename: Wei-Feng
authors.initials: WF
authors.lastname: Wu
authors.forename: Kai
authors.initials: K
authors.lastname: Huang
authors.forename: Jian-Jun
authors.initials: JJ
authors.lastname: Li
authors.forename: Jian-Guo
authors.initials: JG
authors.lastname: He
mesh_terms: D000284:Administration, Oral; D000293:Adolescent; D000328:Adult; D000959:Antihypertensive Agents / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D001794:Blood Pressure / Q000187:drug effects*; D006328:Cardiac Catheterization; D002681:China; D004334:Drug Administration Schedule; D004417:Dyspnea / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D005080:Exercise Test; D017079:Exercise Tolerance / Q000187:drug effects; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D008297:Male; D010879:Piperazines / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D011446:Prospective Studies; D011687:Purines / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use; D000068677:Sildenafil Citrate; D013450:Sulfones / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D013997:Time Factors; D016896:Treatment Outcome; D014655:Vascular Resistance / Q000187:drug effects; D014665:Vasodilator Agents / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D055815:Young Adult
chemical_list: D000959:Antihypertensive Agents; D010879:Piperazines; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate