Long-term Extension Study of the Safety and Pharmacokinetics of QCC374 in PAH Patients

Long-term, Open Label, Multicenter, Extension Study to Evaluate the Safety and Tolerability of QCC374 in Patients With Pulmonary Arterial Hypertension (PAH)

This is a long-term open-label safety extension to the Phase 2a study of inhaled QCC374 in adult patients with PAH. This study provides the patients who completed the QCC374X2201 study with the option to continue receiving QCC374. The study will monitor the long-term safety, tolerability and efficacy of QCC374 in patients with PAH.

Inclusion Criteria:

• Written informed consent must be obtained before any assessment is performed.
• Subject was enrolled in the QCC374X2201 study and completed per protocol

Exclusion Criteria:

• Subjects who have started receiving prostacyclin (epoprostenol), prostacyclin analogs (i.e. trepostinil, iloprost, beraprost) or prostacyclin receptor agonists (i.e. selexipag) since the last study drug intake in the QCC374X2201 study.
• Females who are pregnant, or who plan to become pregnant during the study, or who are breastfeeding
• Any known factor or disease that may interfere with treatment compliance or study conduct (i.e. drug or alcohol dependence)
• Subjects who withdrew consent from the study QCC374X2201