OLE study in participants with pulmonary arterial hypertension

Prior treatment

• Initiation of parenteral administration of prostacyclin analogues (eg, TRE, epoprostenol) since the completion of studies INS1009 201, INS1009 202 or other TPIP studies. Initiation of inhaled prostacyclin analogues (eg, TRE [Tyvaso®] or iloprost) and oral prostacyclin analogues (eg, TRE [Orenitram®]) or receptor agonists (eg, selexipag) are permitted if stopped 24 hours prior to the start of study drug administration.
• Active and current symptomatic COVID-19 or previous severe disease and/or hospitalization due to COVID-19
• Participants who experienced any hypersensitivity or adverse drug reaction or were withdrawn early/discontinued in a previous PAH TPIP study, which, in the opinion of the Investigator, could indicate that continued treatment with TPIP may present an unreasonable risk for the participant.

Other illness

• New evidence of thromboembolic disease as assessed by VQ scan, pulmonary angiography, or pulmonary CT scan.

Lab values

• New onset of heart disease including left ventricular ejection fraction (LVEF) ≤ 40% or clinically significant valvular, constrictive, or symptomatic atherosclerotic heart disease (eg, stable angina, myocardial infarction, etc).

Other

• QTcF interval > 480 ms on resting ECG at screening, not including participants with right bundle branch block (RBBB) leading to a prolongation of the QRS.
• Any new ventricular or supraventricular tachyarrhythmia except for paroxysmal atrial fibrillation and any new symptomatic bradycardia.
• Current use of cigarettes (as defined by CDC) or e-cigarettes: An adult who has smoked at least 100 cigarettes in his or her lifetime, who smokes either every day or some days
• Participants who currently inhale marijuana (recreational or medical).

• Participants who completed the end of treatment visit in Study INS1009 201, Study INS1009 202, or any other lead in PAH TPIP study. Participants for whom the OLE study was not available at the time of their completion of the lead-in study are eligible for enrolment within one year of their lead-in end of treatment visit.
• Complete baseline screening assessments to confirm eligibility to participate if more than 30 days have elapsed since the end of the study visit in Study INS1009 201, Study INS1009 202, or any other lead in PAH TPIP in study.
• Capable of giving signed informed consent that includes compliance with the requirements and restrictions listed in the informed consent form (ICF) and in this protocol.