Measuring Outcomes In Patients With Pulmonary Arterial Hypertension Not on Active Treatment (MOTION)

Inclusion Criteria:

- Male or female patients, 18 to 80 years of age at Visit 0

- Women of childbearing potential must have a negative pre-treatment pregnancy test,
negative monthly pregnancy test, and must use reliable methods of contraception
according to the Risk Evaluation Mitigation Strategies (REMS) guidance

- Symptomatic pulmonary arterial hypertension, World Health Organization Group 1 with a
pulmonary vascular resistance (PVR) >300 dyn*sec*cm-5, mean pulmonary artery pressure
(PAP mean) ≥25 mmHg, and pulmonary capillary wedge pressure (PCWP) ≤15 mmHg as
assessed by right heart catheterization within 6 months prior to Screening (Visit 0)

- PAH of the following types:

Idiopathic (IPAH) Familial (FPAH)

Associated with PAH (APAH) due to:

Connective tissue disease Congenital heart disease, but only if patient underwent surgical
repair more than one year before enrollment Anorexigen or amphetamine use Portal
hypertension with liver cirrhosis

- Not treated with PAH-specific pulmonary medications within 14 days of Screening (Visit
0)

- 6MWD between 150 meters and 450 meters

Exclusion Criteria:

- Patients who are pregnant

- Patients currently on nitrate and/or nitric oxide (NO) donor therapy; patients
currently taking phosphodiesterase 5 (PDE5) inhibitors (such as sildenafil, tadalafil,
vardenafil) and non-specific PDE inhibitors (theophylline, dipyridamole)

- Non-WHO group 1 Pulmonary Hypertension

- Severe restrictive lung disease

- History of uncontrolled high blood pressure or hypotension

- A medical disorder, condition, or history that in the opinion of the Investigator
would impair their ability to participate or complete this study

- Active state of hemoptysis or pulmonary hemorrhage, including those events managed by
bronchial artery embolization