An Open-Label ProSpective MultiCENTer Study to Evaluate Safety and Tolerability of Dry Powder Inhaled Treprostinil in PH

Criteria the registry did not label

• Cohort A Key Inclusion Criteria:
• Males or Females between 18 years to 80 years of age.
• Has a confirmed diagnosis of WHO Group 3 PH-ILD based on CT chest imaging performed, which demonstrates evidence of diffuse parenchymal lung disease and FEV1/FVC (absolute values) ≥ 70% and are required to have evidence of pulmonary hypertension (PH) as demonstrated from right heart catheterization (RHC) with the following hemodynamic parameters.
  • i) Pulmonary vascular resistance (PVR) ≥ 3 Wood Units (WU) and ii) Pulmonary capillary wedge pressure (PCWP) of ≤ 15 mmHg and iii) A mean pulmonary arterial pressure (mPAP) of ≥ 30 mmHg.
  • OR
  • An exploratory subset of subjects with ILD: i) Pulmonary vascular resistance (PVR) ≥ 3 Wood Units (WU) and ii) Pulmonary capillary wedge pressure (PCWP) of ≤ 15 mmHg and iii) A mean pulmonary arterial pressure (mPAP) of ≥ 21 mmHg.
• 6-minute walk distance of ≥ 125 meters
  • Cohort A Key Exclusion Criteria:
  • A Subject is not eligible for inclusion in the study if any of the following criteria apply:
• PH in the Updated WHO Classification Groups 1, 2, 4, or 5.
• History of hemodynamically significant left-sided heart disease.
• Exacerbation of underlying lung disease or active pulmonary or upper respiratory infections.
• Initiation of pulmonary rehabilitation.
  • Cohort B Key Inclusion Criteria
• Male or Females between 18 years to 75 years of age at Screening.
• Has a diagnosis of WHO Group 3 PH-ILD confirmed with CT chest imaging performed at the screening visit, which demonstrates evidence of diffuse parenchymal lung disease and FEV1/FVC (absolute values) ≥70%. Subjects are required to have evidence of pulmonary hypertension (PH) as demonstrated from right heart catheterization (RHC) with the following documented parameters:
  • Pulmonary vascular resistance (PVR) ≥3 Wood Units (WU) and
  • Pulmonary capillary wedge pressure (PCWP) of ≤ 15 mmHg and
  • A mean pulmonary arterial pressure (mPAP) of ≥ 25 mmHg
• Subjects must be on protocol specified dose of inhaled treprostinil QID not at treatment goal, and able to transition from their prescribed dose of inhaled Treprostinil therapy to LIQ861.
• 6-minute walk distance of ≥ 200 meters
  • Cohort B Key Exclusion Criteria
• PH in the Updated WHO Classification Groups 1, 2, 4, or 5.
• Inability to titrate inhaled treprostinil above 5 breaths (Tyvaso®) or above 16 mcg Tyvaso DPI®.
• History of Bronchospasm with Tyvaso or Tyvaso DPI.
• History of persistent moderate asthma or severe asthma.
• History of hemodynamically significant left-sided heart disease.
• Exacerbation of underlying lung disease or active pulmonary or upper respiratory infections.