Early Intervention With Inhaled Treprostinil Among Patients With Exercise-Induced Pulmonary Hypertension (EPIH)

NCT07837830 | Recruiting Early Phase 1 FDA Drug

• 1 other identifier: 01.26_TVH
• Study Type: interventional
• Target: 32
• Locations: 1 country
• Sites: 2

Brief 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. 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.

Conditions

Pulmonary Hypertension

Keywords

Pulmonary Hypertension; Treprostinil; ILD; Pulmonary Vascular Resistance; Pulmonary Vascular Disease; iCPET; Connective Tissue Disease

Outcome Measures

Primary Outcomes (1)

• 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

  Baseline to Month 6

Secondary Outcomes (10)

• Incidence of Adverse Events and Treatment Discontinuations

  Through Month 6

• Progression to Resting Pulmonary Hypertension

  Month 12

• Change in Exercise Pulmonary Vascular Resistance (PVR) at Month 12

  Month 12

• Change in Right Ventricular Stroke Work Index (RVSWI) at Month 6

  Baseline to Month 6

• Change in Pulmonary Arterial Compliance at Month 6

  Baseline to Month 6

Study Arms (2)

Inhaled Treprostinil Plus Standard of Care

EXPERIMENTAL

Participants receive inhaled treprostinil (Tyvaso) in addition to standard disease-specific care.

Drug: Treprostinil

Standard of Care

ACTIVE COMPARATOR

Participants receive standard disease-specific care without inhaled treprostinil

Other: Standard of Care

Interventions

Treprostinil DRUG

Description: * Initial dose 18 mcg four times daily * Titrated to 54-72 mcg four times daily as tolerated Standard disease-specific therapy continues

Inhaled Treprostinil Plus Standard of Care

Standard of Care OTHER

Standard management of ILD/CTD without treprostinil.

Standard of Care

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)

You may qualify if:

• Age ≥18 years
• ILD diagnosis or CTD diagnosis
• WHO Functional Class II-III
• Resting RHC:
• mPAP ≤20 mmHg
• PVR ≤2 WU
• PAWP ≤15 mmHg
• Exercise-induced pulmonary hypertension on iCPET
• Able to provide informed consent

You may not qualify if:

• Resting pulmonary hypertension
• Significant left heart disease
• CTEPH
• Acute PE within 6 months
• Current PAH-targeted therapy
• Pregnancy or breastfeeding
• Severe renal or hepatic impairment
• Inability to perform exercise testing
• Contraindication to RHC
• Treprostinil hypersensitivity

Sponsors & Collaborators

• Valley Health System: lead

Study Sites (2)

The Valley Hospital

Paramus, New Jersey, 07652, United States

RECRUITING

Valley Medical Group Pulmonary Hypertension Clinic

Paramus, New Jersey, 07652, United States

RECRUITING

MeSH Terms

Conditions

Hypertension, Pulmonary; Connective Tissue Diseases

Interventions

treprostinil; Standard of Care

Condition Hierarchy (Ancestors)

Lung Diseases; Respiratory Tract Diseases; Hypertension; Vascular Diseases; Cardiovascular Diseases; Skin and Connective Tissue Diseases

Intervention Hierarchy (Ancestors)

Quality Indicators, Health Care; Quality of Health Care; Health Services Administration; Health Care Quality, Access, and Evaluation

Study Officials

• Ashish Rai Rai, MD

  Valley Health System

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Ashish Rai, MD

CONTACT

201-447-8019; Raias@valleyhealth.com

Emma Goebel, APN

CONTACT

2014478019; nichem@Valleyhealth.com

Study Design

• Study Type: interventional
• Phase: early phase 1
• Allocation: NON RANDOMIZED
• Masking: NONE
• Purpose: TREATMENT
• Intervention Model: PARALLEL
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Model Details: 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

Study Record Dates

• First Submitted: September 10, 2026
• First Posted: September 24, 2026
• Study Start: September 1, 2026
• Primary Completion (Estimated): December 1, 2027
• Study Completion (Estimated): June 1, 2028
• Last Updated: September 24, 2026

Record last verified: 2026-09

Data Sharing

• IPD Sharing: Will not share

Locations

US (2)