A Study of Treprostinil to Treat Interstitial Lung Disease Pulmonary Hypertension

Sponsor

Mayo Clinic (Other)

Overall Status

Recruiting

CT.gov ID

NCT05564637

Collaborator

United Therapeutics (Industry)

Enrollment

Location

Arms

Anticipated Duration (Months)

1.8

Patients Per Site Per Month

Study Details

Study Description

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.

Condition or Disease	Intervention/Treatment	Phase
Pulmonary Arterial Hypertension Interstitial Lung Disease Healthy Adults	Drug: Treprostinil Diagnostic Test: Right Heart Catheterization (RHC) while exercising Diagnostic Test: Optional Muscle Biopsy	Phase 2

Study Design

Study Type:

Interventional

Anticipated Enrollment :

18 participants

Allocation:

Non-Randomized

Intervention Model:

Parallel Assignment

Masking:

None (Open Label)

Primary Purpose:

Treatment

Official Title:

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

Anticipated Study Start Date :

Jan 1, 2023

Anticipated Primary Completion Date :

Nov 1, 2023

Anticipated Study Completion Date :

Nov 1, 2023

Arms and Interventions

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.	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. 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.

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.

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.

Diagnostic Test: Right Heart Catheterization (RHC) while exercising

Diagnostic Test: Optional Muscle Biopsy

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

Outcome Measures

Primary Outcome Measures

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

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 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 resting pulmonary artery pressure [Baseline; after 30 minutes of rest]
Resting pulmonary artery pressure (mmHg) will be measured during the RHC procedure
Change in exercise pulmonary artery pressure [Baseline; approximately 35 minutes]
Exercise 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 exercise right atrial pressure [Baseline; approximately 35 minutes]
Exercise right atrial pressure (mmHg) will be measured during the RHC procedure

Eligibility Criteria

Criteria

Ages Eligible for Study:

18 Years and Older

Sexes Eligible for Study:

All

Accepts Healthy Volunteers:

Yes

Inclusion Criteria:

≥ 18 Years of Age
Patients with suspected Pulmonary Arterial Hypertension related to Interstitial Lung Disease (ILD-PAH) undergoing right heart catheterization.
Undergoing cardiac catheterization for clinical indications at Mayo Clinic Rochester over a 12-month period.
Healthy volunteers will have no known lung disease, heart failure, muscular disease or pulmonary hypertension