Condition: Pulmonary hypertension


Key Inclusion Criteria

  • At least 18 years old
  • Females must not be pregnant or lactating; males and females must use contraception
  • Confirmed diagnosis of World Health Organization (WHO) Group 3 pulmonary hypertension

Key Exclusion Criteria

  • Diagnosis of pulmonary arterial hypertension (PAH) or PH for reasons other than WHO Group 3 PH-interstitial lung disease
  • Intolerance or significant lack of response to a prostacyclin or prostacyclin equivalent
  • Received any PAH approved therapy within 60 days of assignment to study group

Full Study Name

A Multicenter, Randomized, Double-Blinded, Placebo-Controlled Trial to Evaluate the Safety and Efficacy of Inhaled Treprostinil in Subjects With Pulmonary Hypertension Due to Parenchymal Lung Disease (IRB no. 42712)


The purpose of this study is to evaluate the safety of a drug called treprostinil (Tyvaso) and the effectiveness of inhaled treprostinil in patients with pulmonary hypertension (PH) associated with interstitial lung disease (ILD) or combined pulmonary fibrosis and emphysema (CPFE). Researchers will examine the participant's ability to exercise after taking treprostinil.

Participants will be randomly assigned to receive inhaled treprostinil or placebo (inactive substance). The use of inhaled treprostinil in this study is investigational; the drug is approved by the U.S. Food and Drug Administration for pulmonary arterial hypertension but is not approved for PH associated with ILD or CPFE.

Pulmonary hypertension is a condition in which the blood vessels going from the right side of the heart to the lungs (pulmonary arteries) become narrow, putting strain on the heart. Interstitial lung disease causes scarring of the lung, affecting the ability to breathe. Combined pulmonary fibrosis and emphysema is a condition in which both pulmonary fibrosis (scarring of the lungs) and emphysema (damaged air sacs) are present.

Financial disclosure: Victor Tapson, MD, co-investigator for this study, receives payment from the company sponsoring this study. He has received less than $5,000 for consulting work performed for the sponsor. The PI and institution have no other potential financial conflict of interest with respect to this study.


Phase II-III

Principal Investigator

Antoine Hage, MD

Jack Haslett
Phone: 310-248-7141
Email: jack.haslett@cshs.org