Oral sildenafil treatment for Eisenmenger syndrome: a prospective, open-label, multicentre study

BackgroundAlthough 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.ObjectiveTo investigate whether long-term treatment (12 months) with the phosphodiesterase type 5 inhibitor sildenafil improves clinical and haemodynamic parameters in patients with Eisenmenger syndrome.DesignProspective, open-label, multicentre study.SettingFour pulmonary hypertension centres in China.Patients84 Eisenmenger syndrome functional class II–IV patients.InterventionsOral sildenafil 20 mg orally three times a day.Outcome measures6-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.ResultsThe overall treatment effects at 12 months versus baseline (mean changes with 95% CIs) were 56 m increase (42 to 69, p