A Study of Treprostinil to Treat Interstitial Lung Disease Pulmonary Hypertension

Comprehensive O2 Transfer Analysis From the Lung to Mitochondria of Inhaled Treprostinil in Interstitial Lung Disease Pulmonary Hypertension

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.

Study Overview

• Status: Completed
• Conditions: Pulmonary Arterial Hypertension; Interstitial Lung Disease; Healthy Adults
• Intervention / Treatment: Diagnostic test: Right Heart Catheterization (RHC) while exercising; Diagnostic test: Optional Muscle Biopsy; Drug: Treprostinil

• Study Type: Interventional
• Enrollment (Actual): 29
• Phase: Phase 2

Contacts and Locations

Study Locations

• United States
  • Minnesota
    • Rochester, Minnesota, United States, 55905
      • Mayo Clinic in Rochester

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: Yes

Description

Inclusion Criteria:

• 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.

Exclusion Criteria:

• Inability to exercise
• Females who are pregnant

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Treatment
• Allocation: Non-Randomized
• Interventional Model: Parallel Assignment
• Masking: None (Open Label)

Number of Arms

2

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Other: Healthy Volunteers; Healthy volunteers will undergo RHC while exercising and an optional muscle biopsy.	Diagnostic test: Right Heart Catheterization (RHC) while exercising; 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.; Diagnostic test: Optional Muscle Biopsy; A sample of muscle tissue will be collected and analyzed for the ability of the cells to utilize oxygen.
Experimental: PAH-ILD Patients; Patients diagnosed with PAH-ILD will undergo RHC while exercising, receive inhaled treprostinil, and undergo an optional muscle biopsy.	Diagnostic test: Right Heart Catheterization (RHC) while exercising; 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.; Diagnostic test: Optional Muscle Biopsy; A sample of muscle tissue will be collected and analyzed for the ability of the cells to utilize oxygen.; Drug: Treprostinil; 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.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
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 Outcome Measures

Outcome Measure	Measure Description	Time Frame
Ventilatory Efficiency (VE/VCO2)	Ventilatory efficiency was assessed as a ratio between the ventilation (VE) and carbon dioxide production (VCO2) measured during the RHC procedure. VE/VCO2 evaluates how efficiently a person breathes during exercise.	Baseline, approximately 35 minutes
New York Heart Association (NYHA) Functional Class	Participants severity of heart failure symptoms was classified according to the NHYA functional class (I-IV). Lower classifications indicate lower severity of heart failure symptoms.	Baseline, 3-months
Resting Pulmonary Vascular Resistance	Resting pulmonary vascular resistance was measured in Wood units (mm Hg/L/min) during the RHC procedure. Resting pulmonary vascular resistance is the resistance against blood flow from the pulmonary veins to the left atrium of the heart.	Baseline; after 30 minutes of rest
Exercise Pulmonary Vascular Resistance	Exercise pulmonary vascular resistance was measured in Wood units (mm Hg/L/min) during the RHC procedure.	Baseline; approximately 35 minutes
Resting Pulmonary Artery Pressure	Resting pulmonary artery pressure (mmHg) was measured during the RHC procedure. Pulmonary artery pressure is the pressure within the pulmonary arteries, which transport deoxygenated blood from the right side of the heart to the lungs for oxygenation.	Baseline; after 30 minutes of rest
Exercise Pulmonary Artery Pressure	Exercise Pulmonary artery pressure (mmHg) was measured during the RHC procedure. Pulmonary artery pressure is the pressure within the pulmonary arteries, which transport deoxygenated blood from the right side of the heart to the lungs for oxygenation.	Baseline; approximately 35 minutes
Resting Right Atrial Pressure	Resting right atrial pressure (mmHg) was measured during the RHC procedure. Right atrial pressure is a measurement that reflects the amount of blood returning to the heart and the heart's ability to pump blood into the arterial system.	Baseline; after 30 minutes of rest
Exercise Right Atrial Pressure	Exercise right atrial pressure (mmHg) was measured during the RHC procedure. Right atrial pressure is a measurement that reflects the amount of blood returning to the heart and the heart's ability to pump blood into the arterial system.	Baseline; approximately 35 minutes

Collaborators and Investigators

• Sponsor: Mayo Clinic
• Collaborators: United Therapeutics
• Investigators: Principal Investigator: Yogesh N Reddy, M.B.B.S, Mayo Clinic

Publications and helpful links

Helpful Links

• Mayo Clinic Clinical Trials

Study record dates

Study Major Dates

• Study Start (Actual): March 13, 2023
• Primary Completion (Actual): October 30, 2024
• Study Completion (Actual): October 30, 2024

Study Registration Dates

• First Submitted: September 28, 2022
• First Submitted That Met QC Criteria: September 29, 2022
• First Posted (Actual): October 3, 2022

Study Record Updates

• Last Update Posted (Estimated): December 3, 2025
• Last Update Submitted That Met QC Criteria: November 19, 2025
• Last Verified: November 1, 2025

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Respiratory Tract Diseases; Lung Diseases; Hypertension, Pulmonary; Pulmonary Arterial Hypertension; Lung Diseases, Interstitial; Antihypertensive Agents; treprostinil
• Other Study ID Numbers: 22-004971

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: Yes
• Studies a U.S. FDA-regulated device product: No
• product manufactured in and exported from the U.S.: Yes