Long-Term Extension Study of Inhaled Nitric Oxide (iNO) for PAH

An open-label, long-term study to evaluate the safety of inhaled nitric oxide (iNO) in
subjects with pulmonary arterial hypertension (PAH) who participated in IK-7001-PAH-201 and
to provide these patients with continued access to chronic iNO until the time of approval or
when the trial is discontinued due to lack of efficacy.

Inclusion Criteria:

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

- PAH subjects who have completed all EOS assessments in IK-7001-PAH-201 study and have
continued drug/device usage until EOS.

- Subjects are willing and considered in the judgement of the Investigator able to use
the INOpulse device for up to 24 hours per day and could benefit from continued use.

- All female subjects must be willing to continue to take adequate precaution to avoid
pregnancy by using suitable methods of birth control.

Exclusion Criteria:

- Subjects who require treatment with riociguat

- Subjects who early discontinued drug/device usage due to withdrawal of consent or an
AE requiring termination from treatment in IK-7001-PAH-201