title: The efficacy and safety of sildenafil in patients with pulmonary arterial hypertension associated with the different types of congenital heart disease.
abstract: BACKGROUND
  The difference in underlying pathophysiology in different congenital heart disease (CHD) may have an influence on clinical outcome. It remains unclear whether the effect of sildenafil on pulmonary arterial hypertension (PAH) varies in different types of CHD.
  
  
  OBJECTIVE
  The potential effect of sildenafil on pulmonary arterial hypertension related to CHD may be associated with shunt location.
  
  
  METHODS
  In this 12-week, prospective, open label, multicenter trial, 55 patients with CHD were divided into the 3 groups: atrial septal defects group (ASD, n = 15), ventricular septal defects group (VSD, n = 24), and patent ductus arteriosus group (PDA, n = 16). Exercise capacity, hemodynamic parameters, and arterial oxygen saturation were assessed at baseline and after sildenafil therapy (25 mg, 3 times daily).
  
  
  RESULTS
  Six-minute walk distance significantly increased from 377.2 ± 68.7 m to 436.0 ± 70.4 m in patients with ASD, from 371.2 ± 66.0 m to 413.7 ± 83.1 m in VSD, and from 384.3 ± 90.2 m to 440.9 ± 71.8 m in PDA (P<0.01, respectively). Moreover, sildenafil also improved the pulmonary vascular resistance and pulmonary blood flow index in the 3 groups, whereas no significant changes in systemic vascular resistance and systemic arterial pressure were observed. However, arterial oxygen saturation was significantly improved in the ASD group only. The incidence of adverse events was similar among the 3 groups.
  
  
  CONCLUSIONS
  Sildenafil therapy seems to be effective and safe for PAH secondary to ASD, VSD, and PDA, although some clinical and hemodynamic parameters were changed in a different manner among the 3 groups.
authors.affiliation: Center for Diagnosis and Management of Pulmonary Vascular Diseases, Department of Cardiology, Cardiovascular Institute, Fu Wai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
authors.forename: Wei-Jie
authors.initials: WJ
authors.lastname: Zeng
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: Zhi-Hong
authors.initials: ZH
authors.lastname: Liu
authors.forename: Xin-Hai
authors.initials: XH
authors.lastname: Ni
authors.forename: Qing
authors.initials: Q
authors.lastname: Gu
authors.forename: Zhi-Hui
authors.initials: ZH
authors.lastname: Zhao
authors.forename: Jian-Jun
authors.initials: JJ
authors.lastname: Li
authors.forename: Jian-Guo
authors.initials: JG
authors.lastname: He
mesh_terms: D000293:Adolescent; D000328:Adult; D000959:Antihypertensive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D016009:Chi-Square Distribution; D002681:China; D004374:Ductus Arteriosus, Patent / Q000150:complications / Q000503:physiopathology; D017079:Exercise Tolerance / Q000187:drug effects; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006330:Heart Defects, Congenital / Q000097:blood / Q000150:complications* / Q000503:physiopathology; D006344:Heart Septal Defects, Atrial / Q000150:complications / Q000503:physiopathology; D006345:Heart Septal Defects, Ventricular / Q000150:complications / Q000503:physiopathology; D006439:Hemodynamics / Q000187:drug effects; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D008297:Male; D010100:Oxygen / Q000097:blood; D010879:Piperazines / Q000009:adverse effects / Q000627:therapeutic use*; D011446:Prospective Studies; D011687:Purines / Q000009:adverse effects / Q000627:therapeutic use; D000068677:Sildenafil Citrate; D013450:Sulfones / Q000009:adverse effects / Q000627:therapeutic use*; D013997:Time Factors; D016896:Treatment Outcome; D014665:Vasodilator Agents / 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; D010100:Oxygen