A Study of Treprostinil to Treat Interstitial Lung Disease Pulmonary Hypertension

Mayo Clinic29 enrolled

Overview

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 Type

INTERVENTIONAL

Allocation

NON_RANDOMIZED

Purpose

TREATMENT

Masking

NONE

Enrollment

Conditions

Pulmonary Arterial Hypertension Interstitial Lung Disease Healthy Adults

Interventions

TreprostinilDRUG

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.

Right Heart Catheterization (RHC) while exercisingDIAGNOSTIC_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.

Optional Muscle BiopsyDIAGNOSTIC_TEST

Eligibility

Sex: ALLMin age: 18 YearsHealthy volunteers:

Medical Language ↔ Plain English

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

Locations (1)

Mayo Clinic in Rochester

Rochester, Minnesota, United States

Outcomes

Primary Outcomes

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).

Time frame: Baseline, approximately 35 minutes

Secondary Outcomes

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.

Time frame: 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.

Time frame: 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.

Time frame: 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.

Time frame: 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.

Data from ClinicalTrials.gov

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