A Study of Treprostinil to Treat Interstitial Lung Disease Pulmonary Hypertension

NCT05564637 | Completed Phase 2 Results FDA Drug

• 1 other identifier: 22-004971
• Study Type: interventional
• Target: 29
• Locations: 1 country
• Sites: 1

Brief Summary

The purpose of this research study is to find out more about the drug treprostinil via inhaler and the mechanisms of why patients with pulmonary arterial hypertension related to Interstitial Lung disease (PAH-ILD) have limitations during exercise. The investigator is studying treprostinil's effect on patients with PAH-ILD during exercise and its effect on their quality of life after using it for 3 months.

Conditions

Interstitial Lung Disease; Healthy Adults; Pulmonary Arterial Hypertension

Outcome Measures

Primary Outcomes (1)

• Cardiac Output Reserve (Qc)

  Cardiac output reserve (Qc) is the difference between the heart's maximum output during exercise and its resting output. This was measured using the direct Fick method (L/min).

  Baseline, approximately 35 minutes

Secondary Outcomes (8)

• Ventilatory Efficiency (VE/VCO2)

  Baseline, approximately 35 minutes

• New York Heart Association (NYHA) Functional Class

  Baseline, 3-months

• Resting Pulmonary Vascular Resistance

  Baseline; after 30 minutes of rest

• Exercise Pulmonary Vascular Resistance

  Baseline; approximately 35 minutes

• Resting Pulmonary Artery Pressure

  Baseline; after 30 minutes of rest

Study Arms (2)

Healthy Volunteers

OTHER

Healthy volunteers will undergo RHC while exercising and an optional muscle biopsy.

Diagnostic Test: Right Heart Catheterization (RHC) while exercising; Diagnostic Test: Optional Muscle Biopsy

PAH-ILD Patients

EXPERIMENTAL

Patients diagnosed with PAH-ILD will undergo RHC while exercising, receive inhaled treprostinil, and undergo an optional muscle biopsy.

Drug: Treprostinil; Diagnostic Test: Right Heart Catheterization (RHC) while exercising; Diagnostic Test: Optional Muscle Biopsy

Interventions

Optional Muscle Biopsy DIAGNOSTIC_TEST

A sample of muscle tissue will be collected and analyzed for the ability of the cells to utilize oxygen.

Healthy Volunteers; PAH-ILD Patients

Treprostinil DRUG

30mcg (5 breaths) of inhaled treprostinil will be administered during the RHC procedure and patients will be initiated on outpatient inhaled treprostinil therapy for 3-months with gradual up titration to target dose as per standard clinical practice.

PAH-ILD Patients

Right Heart Catheterization (RHC) while exercising DIAGNOSTIC_TEST

A procedure to measure how well or poorly your heart is pumping and to measure the pressures in the heart and lungs. A catheter will be inserted into a vein and artery in order to take the measurements and this will be conducted while exercising on a supine cycle ergometer. Various measurements will be taken during periods of rest and exercise.

Healthy Volunteers; PAH-ILD Patients

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Adult (18-64), Older Adult (65+)

You may qualify if:

• Patients with Pulmonary Arterial Hypertension due to Interstitial Lung Disease (ILD-PAH) being considered for inhaled treprostinil
• Resting right heart catheterization with mean pulmonary artery pressure \>20 mmHg and PVR\> 3 Wood units
• Healthy volunteers will have no known lung disease, heart failure, muscular disease or pulmonary hypertension, no bleeding abnormalities, and have the ability to exercise.

You may not qualify if:

• Inability to exercise
• Females who are pregnant

Sponsors & Collaborators

• Mayo Clinic: lead
• United Therapeutics: collaborator

Study Sites (1)

Mayo Clinic in Rochester

Rochester, Minnesota, 55905, United States

Location

Related Links

• Mayo Clinic Clinical Trials

MeSH Terms

Conditions

Pulmonary Arterial Hypertension; Lung Diseases, Interstitial

Interventions

treprostinil

Condition Hierarchy (Ancestors)

Hypertension, Pulmonary; Lung Diseases; Respiratory Tract Diseases

Results Point of Contact

• Title: Yogesh Reddy, M.B.B.S.
• Organization: Mayo Clinic

reddy.yogesh@mayo.edu

507-284-2904

Study Officials

• Yogesh N Reddy, M.B.B.S

  Mayo Clinic

  PRINCIPAL INVESTIGATOR

Publication Agreements

• PI is Sponsor Employee: Yes

Study Design

• Study Type: interventional
• Phase: phase 2
• Allocation: NON RANDOMIZED
• Masking: NONE
• Purpose: TREATMENT
• Intervention Model: PARALLEL
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Principal Investigator

Study Record Dates

• First Submitted: September 28, 2022
• First Posted: October 3, 2022
• Study Start: March 13, 2023
• Primary Completion: October 30, 2024
• Study Completion: October 30, 2024
• Last Updated: December 3, 2025
• Results First Posted: December 3, 2025

Record last verified: 2025-11

Data Sharing

• IPD Sharing: Will not share

Locations

US (1)