1. Subjects aged 18 and over who have any of the following conditions:
- Idiopathic pulmonary arterial hypertension.
- PAH associated with connective tissue disease (CTD).
- PAH with surgical repair, at least 5yrs previously, of atrial septal defect
(ASD), ventricular septal defect (VSD) patent ductus arteriosis (PDA) or
aorta-pulmonary window.
2. Subjects with a mean pulmonary artery pressure (mPAP) > 25mmHg and a pulmonary artery
wedge pressure (PAWP)
3. Subjects whose baseline 6-min walk test distance was > 100m and
1. PAH secondary to any etiology other than those specified in the inclusion criteria.
2. Subjects with congenital heart disease (other than those specified in the inclusion
criteria), PAH due to thrombo-embolism, HIV, chronic obstructive airways disease,
congestive heart failure.
3. Subjects whose 6-Minute Walk test might have been limited by conditions other than
PAH, associated dyspnea or fatigue.