Pulmonary HypertensionEarly Phase 1EPIH IITNCT07837830
Early Intervention With Inhaled Treprostinil Among Patients With Exercise-Induced Pulmonary Hypertension (EPIH)
A Early Phase 1 study testing Treprostinil in people with Pulmonary Hypertension, run by Valley Health System at 2 sites in United States.
Trial summary
This prospective open-label matched-control pilot study evaluates whether inhaled treprostinil improves exercise pulmonary vascular resistance and functional outcomes in adults with interstitial lung disease or connective tissue disease and exercise-induced pulmonary hypertension. Read more about this study
- Who is it for?
- Ages 18 years and older
- Conditions the study is for
- Pulmonary Hypertension
- What is being tested?
- Treprostinil
- Could I receive a placebo?
- No, everyone receives an active treatment
- How many people
- 32 participants
- Where
- 2 sitesUnited States
- Study dates
- Sep 2026to Jun 2028
- Phase
- Early Phase 1
- Are there results?
- Not yet: the study is under way
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 18 years and older
- All sexes
- No healthy volunteers
- Adult, Older adult
Who may be able to join
- Age ≥18 years
- ILD diagnosis or CTD diagnosis
- WHO Functional Class II-III
- Resting RHC:
- mPAP ≤20 mmHg
- PVR ≤2 WU
Show all 9Show fewer
- PAWP ≤15 mmHg
- Exercise-induced pulmonary hypertension on iCPET
- Able to provide informed consent
Who cannot join
- Resting pulmonary hypertension
- Significant left heart disease
- CTEPH
- Acute PE within 6 months
- Current PAH-targeted therapy
- Pregnancy or breastfeeding
Show all 10Show fewer
- Severe renal or hepatic impairment
- Inability to perform exercise testing
- Contraindication to RHC
- Treprostinil hypersensitivity
Trial locations and contacts
2 sites in 1 country. Contact the study team or a site near you to ask about taking part.
United States2 sites, 2 recruiting
- Recruiting
The Valley Hospital
Paramus, New Jersey, 07652
Ashish Rai, MD, 201-447-8019, Raias@valleyhealth.com
Emma Goebel, APN, 2014478019, nichem@Valleyhealth.com
- Recruiting
Valley Medical Group Pulmonary Hypertension Clinic
Paramus, New Jersey, 07652
About this study
Official title: Early Intervention With Inhaled Treprostinil Among Patients With Exercise-Induced Pulmonary Hypertension: A Prospective Open-Label Matched Control Pilot Trial.
This prospective open-label matched-control pilot study evaluates whether inhaled treprostinil improves exercise pulmonary vascular resistance and functional outcomes in adults with interstitial lung disease or connective tissue disease and exercise-induced pulmonary hypertension. Participants will receive inhaled treprostinil plus standard care or standard care alone and will be followed for 6 months, with an optional extension to 12 months.
Read the detailed description
1.1 Purpose/Specific Aims This pilot study aims to determine whether early treatment of exercise-induced pulmonary hypertension with inhaled Treprostinil can improve pulmonary vascular hemodynamics, preserve cardiopulmonary reserve, and delay progression to overt resting pulmonary hypertension in patients with interstitial lung disease (ILD) or connective tissue disease (CTD).
- Objectives
Primary Objective:
- To evaluate the effect of inhaled treprostinil on exercise pulmonary vascular resistance (PVR) at 6 months in patients with PH-ILD or PH-CTD exercise-induced pulmonary hypertension.
Secondary Objectives:
- To assess the effect of inhaled treprostinil on additional exercise hemodynamic parameters, including mPAP/CO slope, right ventricular stroke work index (RVSWI), and pulmonary arterial compliance at 6 months.
- To evaluate the effect of inhaled treprostinil on functional capacity measured by 6- minute walk distance at 6 months.
- To assess the effect of inhaled treprostinil on exercise tolerance and dyspnea measured by the dyspnea (BORG) questionnaire at 6 months.
- To evaluate the safety and tolerability of inhaled treprostinil in patients with exercise induced PH.
- To assess the proportion of patients progressing to resting PH at 12 months (if the studyextends based on positive 6-month results).
- To evaluate sustained hemodynamic and functional benefits at 12 months (if the study is extended).
Hypotheses / Research Question(s) Treatment with inhaled treprostinil in patients with PH-ILD or PH-CTD with exercise-induced pulmonary hypertension will result in improvement in pulmonary vascular physiology and clinical outcomes. Specifically, inhaled treprostinil administered over 6 months is hypothesized to reduce exercise pulmonary vascular resistance (PVR), improve exercise hemodynamic parameters including mPAP/CO slope, right ventricular stroke work index, and pulmonary arterial compliance, enhance functional capacity as measured by 6-minute walk distance, and improve exercise tolerance and dyspnea as assessed by BORG scores. Inhaled treprostinil is further hypothesized to be safe and well tolerated in this population and may reduce progression to resting pulmonary hypertension, with sustained hemodynamic and functional benefits observed at 12 months among patients continuing therapy
Research Procedures Screening: Resting RHC on all ILD and CTD patients to exclude resting PH.
Baseline: Exercise RHC + iCPET to identify exercise PH and then select eligible patients. Once a matched pair is identified, one participant will be assigned to receive inhaled treprostinil and the other will receive standard of care. Assignment within each matched pair will be determined using a simple allocation method (such as alternating assignment) to reduce bias.
Treatment Period: 6 months with monthly visits (or more if needed) with 6MWT, BNP, safety assessments.
Primary Endpoint: Month 6 exercise RHC + iCPET, functional and quality of life assessments.
Go/No-Go Decision: Extend to 12 months if ≥ 1.0 WU reduction in peak VR, acceptable safety, ≥ 70% completion rate.
- Conditions
- Pulmonary Hypertension
- Being tested
- Treprostinil, Standard of Care
- Keywords
- Pulmonary Hypertension, Treprostinil, ILD, Pulmonary Vascular Resistance, Pulmonary Vascular Disease, iCPET, Connective Tissue Disease
- Medical subject headings
- Hypertension, Pulmonary, Connective Tissue Diseases
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
TreprostinilDrug
Description:
- Initial dose 18 mcg four times daily
- Titrated to 54-72 mcg four times daily as tolerated Standard disease-specific therapy continues
Standard of CareOther
Standard management of ILD/CTD without treprostinil.
| Study group | Role | What they receive |
|---|---|---|
| Inhaled Treprostinil Plus Standard of Care | Experimental | Participants receive inhaled treprostinil (Tyvaso) in addition to standard disease-specific care. Receives: Drug: Treprostinil |
| Standard of Care | Active comparator | Participants receive standard disease-specific care without inhaled treprostinil Receives: Other: Standard of Care |
What is being measured
The primary outcome is the main question the study is designed to answer.
Change in Exercise Pulmonary Vascular Resistance (PVR)
Change in exercise pulmonary vascular resistance (PVR) from baseline to Month 6, measured during exercise right heart catheterization with invasive cardiopulmonary exercise testing (iCPET). The primary analysis will compare the change in exercise PVR between the inhaled treprostinil and standard-of-care groups
Measured: Baseline to Month 6
Secondary outcomes (10)
Incidence of Adverse Events and Treatment Discontinuations
Incidence of adverse events, serious adverse events, treatment discontinuations, and medication tolerability during treatment with inhaled treprostinil.
Measured: Through Month 6
Progression to Resting Pulmonary Hypertension
Proportion of participants who develop resting pulmonary hypertension at Month 12, defined as resting mean pulmonary artery pressure (mPAP) >20 mmHg and pulmonary vascular resistance (PVR) >2 Wood Units measured by right heart catheterization
Measured: Month 12
Change in Exercise Pulmonary Vascular Resistance (PVR) at Month 12
Change in exercise pulmonary vascular resistance (PVR), measured in Wood Units (WU), from baseline to Month 12 among participants continuing in the extension phase.
Measured: Month 12
Change in Right Ventricular Stroke Work Index (RVSWI) at Month 6
Change in right ventricular stroke work index (RVSWI) from baseline to Month 6, measured during exercise right heart catheterization with invasive cardiopulmonary exercise testing (iCPET). The primary analysis will compare the change in RVSWI between the inhaled treprostinil and standard-of-care groups.
Measured: Baseline to Month 6
Change in Pulmonary Arterial Compliance at Month 6
Change in pulmonary arterial compliance from baseline to Month 6, measured during exercise right heart catheterization with invasive cardiopulmonary exercise testing (iCPET). The primary analysis will compare the change in pulmonary arterial compliance between the inhaled treprostinil and standard-of-care groups.
Measured: Baseline to Month 6
Change in 6-Minute Walk Distance at Month 6
Change in 6-minute walk distance (meters) from baseline to Month 6. The analysis will compare the change between the inhaled treprostinil and standard-of-care groups.
Measured: Baseline to Month 6
Change in Dyspnea Severity (Borg Scale) at Month 6
Change from baseline to Month 6 in dyspnea severity measured using the Borg Category Ratio (CR10) Dyspnea Scale. Scores range from 0 to 10, where 0 indicates no breathlessness and 10 indicates maximal breathlessness. Higher scores indicate worse dyspnea.
Measured: Baseline to Month 6
Change in 6-Minute Walk Distance at Month 12
Change in 6-minute walk distance, measured in meters, from baseline to Month 12 among participants continuing in the extension phase.
Measured: Month 12
Change in Mean Pulmonary Artery Pressure/Cardiac Output (mPAP/CO) Slope at Month 6
Change in mPAP/CO slope from baseline to Month 6, measured during exercise right heart catheterization with invasive cardiopulmonary exercise testing (iCPET). The analysis will compare the change between the inhaled treprostinil and standard-of-care groups.
Measured: Baseline to Month 6
Change in Dyspnea Severity (Borg Category Ratio [CR10] Dyspnea Scale) at Month 12
Change from baseline to Month 12 in dyspnea severity measured using the Borg Category Ratio (CR10) Dyspnea Scale. Scores range from 0 to 10, with higher scores indicating worse dyspnea.
Measured: Month 12
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted24 Sep 2026
- Study startSep 2026 (estimated)
- Primary completionDec 2027 (estimated)
- Study completionJun 2028 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Other trials for Pulmonary HypertensionStudies of the same condition, open and under way.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Interventional
- Phase
- Early Phase 1
- Primary purpose
- Treatment
- Allocation
- Non-randomised
- Intervention model
- Parallel groups
- Model description
- Parallel, open-label, matched-control pilot study comparing inhaled treprostinil plus standard of care versus standard of care alone in adults with exercise-induced pulmonary hypertension
- Masking
- None (open label)
- Enrolment
- 32 participants (estimated)
Sponsor and investigators
- Lead sponsor
- Valley Health System
- Sponsor type
- University, hospital or other
- Responsible party
- Sponsor
- Investigators
- Ashish Rai Rai, MD, Principal investigator, Valley Health System
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT07837830
- Sponsor's protocol ID
- 01.26_TVH
- Acronym
- EPIH IIT
- Status last verified
- Sep 2026
- First submitted
- 10 Sep 2026
- Last updated
- 24 Sep 2026
- Results posted
- No
- Data monitoring committee
- No
- FDA-regulated drug
- Yes
- FDA-regulated device
- No
- Individual data sharing
- No: Individual participant data will not be shared. Results will be reported in aggregate and de-identified form to protect participant confidentiality and privacy. Study records will be maintained in accordance with institutional policies
Study at a glance
From the registry record, last updated by the sponsor on 24 Sep 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Early Intervention With Inhaled Treprostinil Among Patients With Exercise-Induced Pulmonary Hypertension: A Prospective Open-Label Matched Control Pilot Trial.
- ClinicalTrials.gov ID
- NCT07837830
- Acronym / sponsor ID
- EPIH IIT / 01.26_TVH
- Conditions
- Pulmonary Hypertension
- Keywords
- Pulmonary Hypertension, Treprostinil, ILD, Pulmonary Vascular Resistance, Pulmonary Vascular Disease, iCPET, Connective Tissue Disease
- Phase
- Early Phase 1
- Study type
- Interventional
- Recruitment status
- Recruiting
- Enrolment
- 32 participants (estimated)
- Lead sponsor
- Valley Health SystemUniversity, hospital or other
- Study start
- Sep 2026 (estimated)
- Primary completion
- Dec 2027 (estimated)
- Study completion
- Jun 2028 (estimated)
- First posted
- 24 Sep 2026 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
This prospective open-label matched-control pilot study evaluates whether inhaled treprostinil improves exercise pulmonary vascular resistance and functional outcomes in adults with interstitial lung disease or connective tissue disease and exercise-induced pulmonary hypertension. Participants will receive inhaled treprostinil plus standard care or standard care alone and will be followed for 6 months, with an optional extension to 12 months.
Detailed description
1.1 Purpose/Specific Aims This pilot study aims to determine whether early treatment of exercise-induced pulmonary hypertension with inhaled Treprostinil can improve pulmonary vascular hemodynamics, preserve cardiopulmonary reserve, and delay progression to overt resting pulmonary hypertension in patients with interstitial lung disease (ILD) or connective tissue disease (CTD).
- Objectives
Primary Objective:
- To evaluate the effect of inhaled treprostinil on exercise pulmonary vascular resistance (PVR) at 6 months in patients with PH-ILD or PH-CTD exercise-induced pulmonary hypertension.
Secondary Objectives:
- To assess the effect of inhaled treprostinil on additional exercise hemodynamic parameters, including mPAP/CO slope, right ventricular stroke work index (RVSWI), and pulmonary arterial compliance at 6 months.
- To evaluate the effect of inhaled treprostinil on functional capacity measured by 6- minute walk distance at 6 months.
- To assess the effect of inhaled treprostinil on exercise tolerance and dyspnea measured by the dyspnea (BORG) questionnaire at 6 months.
- To evaluate the safety and tolerability of inhaled treprostinil in patients with exercise induced PH.
- To assess the proportion of patients progressing to resting PH at 12 months (if the studyextends based on positive 6-month results).
- To evaluate sustained hemodynamic and functional benefits at 12 months (if the study is extended).
Hypotheses / Research Question(s) Treatment with inhaled treprostinil in patients with PH-ILD or PH-CTD with exercise-induced pulmonary hypertension will result in improvement in pulmonary vascular physiology and clinical outcomes. Specifically, inhaled treprostinil administered over 6 months is hypothesized to reduce exercise pulmonary vascular resistance (PVR), improve exercise hemodynamic parameters including mPAP/CO slope, right ventricular stroke work index, and pulmonary arterial compliance, enhance functional capacity as measured by 6-minute walk distance, and improve exercise tolerance and dyspnea as assessed by BORG scores. Inhaled treprostinil is further hypothesized to be safe and well tolerated in this population and may reduce progression to resting pulmonary hypertension, with sustained hemodynamic and functional benefits observed at 12 months among patients continuing therapy
Research Procedures Screening: Resting RHC on all ILD and CTD patients to exclude resting PH.
Baseline: Exercise RHC + iCPET to identify exercise PH and then select eligible patients. Once a matched pair is identified, one participant will be assigned to receive inhaled treprostinil and the other will receive standard of care. Assignment within each matched pair will be determined using a simple allocation method (such as alternating assignment) to reduce bias.
Treatment Period: 6 months with monthly visits (or more if needed) with 6MWT, BNP, safety assessments.
Primary Endpoint: Month 6 exercise RHC + iCPET, functional and quality of life assessments.
Go/No-Go Decision: Extend to 12 months if ≥ 1.0 WU reduction in peak VR, acceptable safety, ≥ 70% completion rate.
Study design
- Primary purpose
- Treatment
- Allocation
- Non-randomised
- Intervention model
- Parallel groups
- Masking
- None (open label)
- Number of arms
- 2
- Model description
- Parallel, open-label, matched-control pilot study comparing inhaled treprostinil plus standard of care versus standard of care alone in adults with exercise-induced pulmonary hypertension
| Arm | Type | Receives |
|---|---|---|
| Inhaled Treprostinil Plus Standard of Care | Experimental | Drug: TreprostinilParticipants receive inhaled treprostinil (Tyvaso) in addition to standard disease-specific care. |
| Standard of Care | Active comparator | Other: Standard of CareParticipants receive standard disease-specific care without inhaled treprostinil |
Who may qualify
- Ages
- 18 years and older
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Adult, Older adult
Key inclusion criteria (9 listed)
- Age ≥18 years
- ILD diagnosis or CTD diagnosis
- WHO Functional Class II-III
- Resting RHC:
- Exercise-induced pulmonary hypertension on iCPET
Key exclusion criteria (10 listed)
- Resting pulmonary hypertension
- Significant left heart disease
- CTEPH
- Acute PE within 6 months
- Current PAH-targeted therapy
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| TreprostinilDescription: * Initial dose 18 mcg four times daily * Titrated to 54-72 mcg four times daily as tolerated Standard disease-specific therapy continues | Drug | Inhaled Treprostinil Plus Standard of Care |
| Standard of CareStandard management of ILD/CTD without treprostinil. | Other | Standard of Care |
Primary outcome
Change in Exercise Pulmonary Vascular Resistance (PVR)
Time frame: Baseline to Month 6
Change in exercise pulmonary vascular resistance (PVR) from baseline to Month 6, measured during exercise right heart catheterization with invasive cardiopulmonary exercise testing (iCPET). The primary analysis will compare the change in exercise PVR between the inhaled treprostinil and standard-of-care groups
10 secondary outcomes
Incidence of Adverse Events and Treatment Discontinuations
Time frame: Through Month 6
Progression to Resting Pulmonary Hypertension
Time frame: Month 12
Change in Exercise Pulmonary Vascular Resistance (PVR) at Month 12
Time frame: Month 12
Change in Right Ventricular Stroke Work Index (RVSWI) at Month 6
Time frame: Baseline to Month 6
Change in Pulmonary Arterial Compliance at Month 6
Time frame: Baseline to Month 6
Change in 6-Minute Walk Distance at Month 6
Time frame: Baseline to Month 6
Change in Dyspnea Severity (Borg Scale) at Month 6
Time frame: Baseline to Month 6
Change in 6-Minute Walk Distance at Month 12
Time frame: Month 12
Change in Mean Pulmonary Artery Pressure/Cardiac Output (mPAP/CO) Slope at Month 6
Time frame: Baseline to Month 6
Change in Dyspnea Severity (Borg Category Ratio [CR10] Dyspnea Scale) at Month 12
Time frame: Month 12
Results and safety
This study is still under way. Results are not available in the source record yet.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 24 Sep 2026.
- Individual participant dataSharing planned: No. Individual participant data will not be shared. Results will be reported in aggregate and de-identified form to protect participant confidentiality and privacy. Study records will be maintained in accordance with institutional policies
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part.
Ashish Rai Rai, MD principal investigator
Valley Health System
2 sites in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
A Early Phase 1 study testing Treprostinil in people with Pulmonary Hypertension, run by Valley Health System at 2 sites in United States.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 9 inclusion and 10 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: looking for participants now, as of the sponsor's last update on 24 Sep 2026. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 2 sites in United States. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
What does this phase mean?
Early Phase 1 (sometimes called Phase 0) studies give a very small amount of a treatment to a few people, mainly to see how the body handles it.
Not every study follows the same path, and some studies (observational studies, device studies) have no phase.
Are results available for this study?
This study is still under way. Results are not available in the source record yet.
How can a doctor refer a patient or contact the research team?
Contact Ashish Rai, MD (201-447-8019, Raias@valleyhealth.com), or a site near the patient. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Change in Exercise Pulmonary Vascular Resistance (PVR) (time frame: Baseline to Month 6). It is what the study was designed to measure, not a finding.
Where can I read the original study information?
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 24 Sep 2026.