A Study of Treprostinil to Treat Interstitial Lung Disease Pulmonary Hypertension

Phase 2

Completed

• Conditions: Pulmonary Arterial Hypertension; Interstitial Lung Disease; Healthy Adults
• Interventions: Drug: Treprostinil; Diagnostic Test: Optional Muscle Biopsy; Diagnostic Test: Right Heart Catheterization (RHC) while exercising

• Registration Number: NCT05564637
• Lead Sponsor: Mayo Clinic

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.

Detailed Description

Not available

Study Timeline

• Study First Submitted: 2022-09-28
• Study First Submitted QC: 2022-09-29
• Study First Posted: 2022-10-03
• Study Start: 2023-03-13
• Primary Completion: 2024-10-30
• Study Completion: 2024-10-30
• Status Verified: 2025-02
• Last Update Submitted: 2025-02-03
• Last Update Posted: 2025-02-04

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 29

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 & Design

• Study Type: INTERVENTIONAL
• Study Design: PARALLEL

Arm && Interventions

Group	Intervention	Description
Healthy Volunteers	Optional Muscle Biopsy	Healthy volunteers will undergo RHC while exercising and an optional muscle biopsy.
PAH-ILD Patients	Right Heart Catheterization (RHC) while exercising	Patients diagnosed with PAH-ILD will undergo RHC while exercising, receive inhaled treprostinil, and undergo an optional muscle biopsy.
Healthy Volunteers	Right Heart Catheterization (RHC) while exercising	Healthy volunteers will undergo RHC while exercising and an optional muscle biopsy.
PAH-ILD Patients	Optional Muscle Biopsy	Patients diagnosed with PAH-ILD will undergo RHC while exercising, receive inhaled treprostinil, and undergo an optional muscle biopsy.
PAH-ILD Patients	Treprostinil	Patients diagnosed with PAH-ILD will undergo RHC while exercising, receive inhaled treprostinil, and undergo an optional muscle biopsy.

Primary Outcome Measures

Name	Time	Method
Change in cardiac output reserve (Qc)	Baseline, approximately 35 minutes	Cardiac output reserve (Qc) will be measured using the direct Fick method (L/min) during the RHC procedure.

Secondary Outcome Measures

Name	Time	Method
Change in exercise pulmonary artery pressure	Baseline; approximately 35 minutes	Exercise Pulmonary artery pressure (mmHg) will be measured during the RHC procedure
Change in ventilatory efficiency (VE/VCO2)	Baseline, approximately 35 minutes	Ventilatory efficiency will be assessed as a ratio between the ventilation (VE) and carbon dioxide production (VCO2) measured during the RHC procedure.
Change in resting pulmonary artery pressure	Baseline; after 30 minutes of rest	Resting pulmonary artery pressure (mmHg) will be measured during the RHC procedure
Change in resting right atrial pressure	Baseline; after 30 minutes of rest	Resting right atrial pressure (mmHg) will be measured during the RHC procedure
Change in New York Heart Association (NYHA) functional class	Baseline, 3-months	Patients severity of heart failure symptoms will be classified according to the NHYA functional class (I-IV). Lower classifications indicate lower severity of heart failure symptoms.
Change in resting pulmonary vascular resistance	Baseline; after 30 minutes of rest	Resting pulmonary vascular resistance will be measured in Wood units (mm Hg/L/min) during the RHC procedure
Change in exercise pulmonary vascular resistance	Baseline; approximately 35 minutes	Exercise pulmonary vascular resistance will be measured in Wood units (mm Hg/L/min) during the RHC procedure
Change in exercise right atrial pressure	Baseline; approximately 35 minutes	Exercise right atrial pressure (mmHg) will be measured during the RHC procedure

Trial Locations

Locations (1)

Mayo Clinic in Rochester; 🇺🇸 Rochester, Minnesota, United States