Effects of Oral Sildenafil on Mortality in Adults With PAH
Effects of Oral Sildenafil on Mortality in Adults With PAH
This is a blinded study in adult patients with PAH evaluating the relative effects of
 sildenafil on mortality when administered at the three doses (80 mg, 20 mg or 5 mg, all three
 times per day [TID]). In addition, the relative effects on clinical worsening and 6-minute
 walking distance (6MWD) will be assessed.
Inclusion Criteria:

 Subjects ≥ 18 <75 years of age with any of the following conditions:

 -  Idiopathic Primary Pulmonary Arterial Hypertension (IPAH)

 -  PAH secondary to connective tissue disease

 -  PAH with surgical repair (at least 5 years previously) of atrial septal defect
 (ASD),ventricular septal defect (VSD), patent ductus arteriosus (PDA) and
 aorto-pulmonary window

 -  PAH diagnosis confirmed by right heart catheterization performed within 12 months
 prior to randomization

 -  Functional Class II-IV; Baseline 6MWD ≥ 50 m.

 Exclusion Criteria:

 -  Significant (ie, >2+) valvular disease other than tricuspid regurgitation or pulmonary
 regurgitation

 -  History of cardiac arrest, respiratory arrest, hemodynamic collapse, CPR, ventricular
 tachycardia, ventricular fibrillation, or uncontrolled atrial fibrillation

 -  History of pulmonary embolism; History of chronic lung disease / restrictive lung
 disease (eg, chronic obstructive pulmonary disease (COPD) or scleroderma) with
 impairment of lung function

 -  No prior long term treatment with PDE-5 inhibitors

 -  Treatment with bosentan OR riociguat within 3 months of randomization

 -  Current treatment with nitrates or nitric oxide