A Phase 3 Open-label, Multicenter Study to Evaluate the Long-term Safety and Tolerability of Inhaled LIQ861 (Treprostinil) in Pulmonary Arterial Hypertension (WHO Group I) Patients This study is in follow-up

The purpose of this study to evaluate the long-term safety and tolerability of inhaled LIQ861 (treprostinil) using a dry powder inhaler.

National Clinical Trial (NCT) identifier on clinicaltrials.gov: NCT03399604

Inova Heart and Vascular Institute
Advanced Lung Disease and Transplant Clinic
3300 Gallows Road, Falls Church, VA 22042

Oksana Shlobin, MD

• Male or female patients =/> 18 years of age
• Diagnosed with PAH belonging to Nice Clinical Classification Grp 1
• NYHA Functional Class II-IV at Screening
• Can complete a baseline 6MWD =/> 150m
• FEV1 =/> 60% and FEV1/FVC ratio =/> 60% during the 6 month period before enrollment

• Diagnosed with PH in the Nice Classification Groups 2-5, or PAH Group 1 subgroups 1.4.3 (associated with portal hypertension) or 1.4.5 (associated with schistosomiasis)
• Patient is currently taking oral prostacyclin analogues or agonists, including treprostinil and selexipag
• Uncontrolled systemic hypertension (=/>160/100 mmHg)
• Has had an atrial septostomy
• Has had a pulmonary infarction within 2 weeks of screening
• Pregnancy
• Has had a stroke or transient ischemic attack within 6 months of screening
• Severe hepatic or renal impairment