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Oral sildenafil treatment for Eisenmenger syndrome: a prospective, open-label, multicentre study
  1. Zhen-Ning Zhang1,2,
  2. Xin Jiang1,
  3. Rui Zhang1,
  4. Xin-Li Li3,
  5. Bing-Xiang Wu4,
  6. Qin-Hua Zhao1,
  7. Yong Wang2,
  8. Li-Zhi Dai1,
  9. Lei Pan2,
  10. Mardi Gomberg-Maitland5,
  11. Zhi-Cheng Jing1
  1. 1Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China
  2. 2Department of Pulmonary Vascular Disease, Beijing Shijitan Hospital, Beijing, China
  3. 3Department of Cardiovascular Medicine, The First Affiliated Hospital of Nanjing Medical University, Nanjing, China
  4. 4Department of Cardiology, The First Affiliated Hospital of Haerbin Medical University, Haerbin, China
  5. 5Pulmonary Hypertension Center, Section of Cardiology, Department of Medicine, Chicago University Medical Center, Chicago, Illinois, USA
  1. Correspondence to Professor Zhi-Cheng Jing, Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, 507, Zhengmin Road, Shanghai 200433, China; jingzhicheng{at}


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.

Design Prospective, open-label, multicentre study.

Setting Four pulmonary hypertension centres in China.

Patients 84 Eisenmenger syndrome functional class II–IV patients.

Interventions Oral sildenafil 20 mg orally three times a day.

Outcome measures 6-min walk distance (6MWD) test, resting systemic arterial blood oxygen saturation (SaO2) 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 SaO2. 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×m2 (−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.

  • Sildenafil
  • phosphodiesterase inhibitors
  • Eisenmenger syndrome
  • pulmonary arterial hypertension
  • pulmonary embolism
  • pulmonary vascular disease
  • deep vein thrombosis
  • congenital heart disease

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  • Z-NZ was formerly a visiting fellow in the Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine. She is now working in the Pulmonary Vascular Department, Beijing Shijitan Hospital, Beijing, China.

  • Z-NZ, XJ and RZ contributed equally to the manuscript. All authors participated in the study, and in the review and approval of the manuscript.

  • Funding The study was sponsored by the Shanghai New Frontier Project (SHDC12010102), the Program for Young Excellent Talents in Tongji University (2009KJ042) and China National 973 Project (2007CB512008).

  • Competing interests Z-CJ has served as a consultant on steering committees for clinical trials and has been a member of scientific advisory boards for Actelion, Bayer-Schering, Pfizer and United Therapeutics; these companies provided funding to Tongji University to support his conduct of clinical trials. MG-M has served as a consultant/participant on data safety monitoring boards/steering committees for clinical trials for Actelion, Gilead, Medtronic and Pfizer. Actelion, Gilead, Lilly/Icos, Pfizer, Novartis and United Therapeutics provided funding to the University of Chicago to support her conduct of clinical trials. None of the other authors has any conflict of interest to declare regarding the content of this paper.

  • Patient consent Obtained.

  • Ethics approval Ethics Committees of all participating centres.

  • Provenance and peer review Not commissioned; externally peer reviewed.