Pulmonary Arterial Hypertension Quality Enhancement Research Initiative Extension Program

To improve the management of PAH patients through an evidence-based approach aimed at
achieving optimal WHO functional class (defined as: 1) improvement of FC III or IV to FC II;
2) improvement of FC II to FC I; or 3) maintaining FC II or I)

Inclusion Criteria:

1. Male and female adults (≥ 18 years of age)

2. Documented diagnosis of PAH (all of the following): i. RHC demonstrating mPAP > 25 mm
Hg and PCWP

≤ 15 and PVR > 3 ii. FEV 1 > 50% predicted normal iii. V/Q and/or CT scan excluding
the thromboembolic etiology

3. Diagnosis of PAH < 3 years

4. Need for PAH specific treatment

5. Desire to participate and signs an informed consent

Exclusion Criteria:

Any of the criteria below:

1. Poor mental function, drug or substance (e.g.,alcohol) abuse, or unstable psychiatric
illness, which, in the opinion of the investigator, may interfere with optimal
participation in the study

2. Prior participation in this program

3. Patients with pulmonary hypertension classified as group 2 - 5 Updated Clinical
Classification of Pulmonary Hypertension (5th World Symposium on PH Modified
Classification of PH)