title: Oral sildenafil treatment for Eisenmenger syndrome: a prospective, open-label, multicentre study.
abstract: BACKGROUND
  Although sildenafil has been shown to be safe and effective in idiopathic pulmonary arterial hypertension (PAH) and PAH related to connective tissue disease, its effects in Eisenmenger syndrome are less clear.
  
  
  OBJECTIVE
  To investigate whether long-term treatment (12 months) with the phosphodiesterase type 5 inhibitor sildenafil improves clinical and haemodynamic parameters in patients with Eisenmenger syndrome.
  
  
  METHODS
  Prospective, open-label, multicentre study.
  
  
  METHODS
  Four pulmonary hypertension centres in China.
  
  
  METHODS
  84 Eisenmenger syndrome functional class II-IV patients.
  
  
  METHODS
  Oral sildenafil 20 mg orally three times a day.
  
  
  METHODS
  6-min walk distance (6MWD) test, resting systemic arterial blood oxygen saturation (SaO(2)) in room air, haemodynamic parameters assessed by right heart catheterisation, safety and tolerability.
  
  
  RESULTS
  The overall treatment effects at 12 months versus baseline (mean changes with 95% CIs) were 56 m increase (42 to 69, p<0.0001) in 6MWD, and 2.4% increase (1.8% to 2.9%, p<0.0001) in resting room air SaO(2). Improvements were also seen in mean pulmonary arterial pressure and pulmonary vascular resistance index (-4.7 mm Hg (-7.5 to -1.9), p=0.001; and -474 dyn×s×cm(-5)×m(2) (-634 to -314), p<0.0001, respectively). Sildenafil was well tolerated. Most adverse events were mild and transient, and occurred in the first 2 weeks of treatment.
  
  
  CONCLUSIONS
  Twelve months of oral sildenafil treatment was well tolerated and appeared to improve exercise capacity, systemic arterial oxygen saturation and haemodynamic parameters in patients with Eisenmenger syndrome.
authors.affiliation: Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, China.
authors.forename: Zhen-Ning
authors.initials: ZN
authors.lastname: Zhang
authors.forename: Xin
authors.initials: X
authors.lastname: Jiang
authors.forename: Rui
authors.initials: R
authors.lastname: Zhang
authors.forename: Xin-Li
authors.initials: XL
authors.lastname: Li
authors.forename: Bing-Xiang
authors.initials: BX
authors.lastname: Wu
authors.forename: Qin-Hua
authors.initials: QH
authors.lastname: Zhao
authors.forename: Yong
authors.initials: Y
authors.lastname: Wang
authors.forename: Li-Zhi
authors.initials: LZ
authors.lastname: Dai
authors.forename: Lei
authors.initials: L
authors.lastname: Pan
authors.forename: Mardi
authors.initials: M
authors.lastname: Gomberg-Maitland
authors.forename: Zhi-Cheng
authors.initials: ZC
authors.lastname: Jing
mesh_terms: D000284:Administration, Oral; D000293:Adolescent; D000328:Adult; D002681:China; D004334:Drug Administration Schedule; D004541:Eisenmenger Complex / Q000175:diagnosis / Q000188:drug therapy*; D017079:Exercise Tolerance; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010879:Piperazines / Q000008:administration & dosage / Q000494:pharmacology / Q000627:therapeutic use*; D011446:Prospective Studies; D011687:Purines / Q000008:administration & dosage / Q000494:pharmacology / Q000627:therapeutic use; D012720:Severity of Illness Index; D000068677:Sildenafil Citrate; D013450:Sulfones / Q000008:administration & dosage / Q000494:pharmacology / Q000627:therapeutic use*; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000008:administration & dosage / Q000494:pharmacology / Q000627:therapeutic use*
chemical_list: D010879:Piperazines; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate