Study of PAH Subjects With LTOT Use That Have Demonstrated Improved Exercise Tolerance With the Use of Inhaled Nitric

Phase 3, Multicenter, Randomized, Double-blind, Placebo Controlled Withdrawal Study of
Pulmonary Arterial Hypertension(PAH) Subjects with LTOT Use that have Demonstrated Improved
Exercise Tolerance with the use of Inhaled Nitric Oxide (INO)

Inclusion Criteria:

1. Signed Informed Consent Form prior to the initiation of any study mandated procedures
or assessments

2. Subjects must be enrolled in the PULSE PAH-004 clinical trial and must have been on
LTOT and on open-label treatment with iNO 75 mcg/kg IBW/hr for at least 4 months

3. Subjects must have achieved ≥ 30 meter improvement in 6MWD after 4, 8 or 12 months of
open-label treatment with iNO 75 mcg/kg IBW/hr as compared to either their PULSE
PAH-004 Week 2 end of Run-in OR End of Study (EOS)in PULSE-PAH-004.

4. Subjects are willing and considered in the judgement of the Investigator able to use
the INOpulse device continuously for up to 24 hours per day

5. Female subjects of childbearing potential must have a negative pregnancy test (serum
or urine) at randomization. All female subjects must use an effective method of birth
control to avoid pregnancy.

Exclusion Criteria:

1. Subjects with episodes of worsening of PAH in the last 30 days prior to PULSE PAH-007
Baseline/Randomization

2. Subjects that experience Pulmonary Rebound in PULSE-PAH-004

3. Change in dose or types of PAH specific therapies in the last 30 days prior to
Baseline/Randomization

4. Subjects who require treatment with riociguat

5. Subjects who early discontinued drug/device usage due to withdrawal of consent or an
adverse event requiring termination from treatment in PULSE PAH-004

6. Women who are pregnant

7. The concurrent use of the INOpulse device with a continuous airway pressure (CPAP),
Bilevel Positive Airway Pressure (BiPAP, or any other positive pressure device.