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DeciPHer-ILD: A Real-world Patient Registry in Group 3 Pulmonary Hypertension Associated With Interstitial Lung Disease (PH-ILD)

RecruitingObservational study

Who can join

Ages 18 and older · All sexes

Full eligibility criteria
Inclusion Criteria:

1. Adults aged 18 years or older
2. Diagnosis of fibrotic ILD based on high-resolution computed tomography imaging, including but not limited to:

   1. Idiopathic interstitial pneumonia, including idiopathic pulmonary fibrosis
   2. Autoimmune ILD
   3. Chronic hypersensitivity pneumonitis
   4. Nonspecific interstitial pneumonia
   5. Occupational lung disease
   6. Combined pulmonary fibrosis and emphysema with fibrosis greater than the extent of emphysema on lung imaging as determined by the Investigator
3. For patients to be eligible for Cohorts 1 to 3: RHC confirmed PH (mean pulmonary artery pressure \>20 mmHg, pulmonary artery wedge pressure

   ≤15 mmHg, pulmonary vascular resistance \>2 WU).
4. For patients to be eligible for Cohort 1, they must not be receiving inhaled treprostinil at Baseline.
5. For patients to be eligible for Cohort 2, they must have initiated

Tyvaso/Tyvaso DPI at 1 of the following time points:

1. Baseline
2. ≤90 days prior to Baseline 6. For patients to be eligible for Cohort 3, they must be receiving Tyvaso/Tyvaso DPI at Baseline and for \>90 days prior to Baseline. 7. For patients to be eligible for Cohort 4: Prior RHC not meeting the definition of PH as described in Inclusion Criterion 3 but has a pulmonary artery wedge pressure ≤15 mmHg. 8. Co-enrollment in other observational or interventional studies is permitted. 9. Patient is willing and able to provide informed consent and complete surveys/questionnaires in English or Spanish.

Exclusion Criteria:

1. Confirmed diagnosis of Group 1, 2, 4, or 5 PH

   a. In cases in which a patient has a diagnosis of PH-ILD along with a concomitant diagnosis of another PH group (eg, Group 1), enrollment may be permitted at the Investigator's discretion if, in their clinical judgment, PH-ILD is the predominant driver of the patient's PH.
2. Confirmed diagnosis of Group 3 PH associated with lung diseases and or hypoxia other than fibrotic ILD, as outlined in Inclusion Criterion 2.
3. Patients receiving inhaled treprostinil (Yutrepia™) at Baseline. Patients receiving other forms of PH therapy will be eligible for enrollment.

About the study

This is a prospective, real world, multicenter, registry of patients with pulmonary hypertension associated with interstitial lung disease (PH-ILD) and interstitial lung disease (ILD).

What is being tested

Sponsor: United Therapeutics · Participants: 900 · Started: Jan 23, 2025

Contact the study team

Official record on ClinicalTrials.gov — NCT06388421

Locations in the U.S.

AlabamaUniversity of Alabama at Birmingham, Birmingham
ArizonaBanner Health - University Medical Center Phoenix, Phoenix
CaliforniaCedars-Sinai Medical Center, Los Angeles
UC Davis Health Medical Center, Sacramento
ColoradoUniversity of Colorado - Anschutz Medical Campus, Aurora
National Jewish Health, Denver
District of ColumbiaPasquerilla Healthcare Center - Georgetown University, Washington D.C.
FloridaUniversity of Florida, Gainesville
NCH Research Institute, Naples
Advent Health Medical Group Advanced Lung Disease, Orlando
Intercoastal Medical Group, Sarasota
Tampa General Hospital/University of South Florida Health, Tampa
Weston Hospital - Cleveland Clinic Florida, Weston
GeorgiaEmory University, Atlanta
Southeastern Cardiology Associates, Columbus
IllinoisNorthwestern University, Chicago
Loyola University Medical Center, Maywood
Advocate Christ Medical Center, Oak Lawn
IndianaAscension St. Vincent, Indianapolis
KansasUniversity of Kansas Medical Center, Kansas City
KentuckyNorton Pulmonary Specialists, Louisville
LouisianaUniversity Medical Center - New Orleans, New Orleans
MarylandUniversity of Maryland Medical Center, Baltimore
MichiganCorewell Health Beamont Troy Hospital, Troy
MinnesotaMayo Clinic - Rochester, Rochester
MissouriWashington University School of Medicine, St Louis
New HampshireDartmouth Hitchcock Medical Center, Lebanon
New JerseyMorristown Medical Center, Morristown
HMH Jersey Shore University Medical Ctr., Neptune City
New MexicoUniversity of New Mexico Health Sciences Center, Albuquerque
New YorkNorthwell Health, New Hyde Park
New York Presbyterian Hospital - Weill Cornell Medicine, New York
New York University Langone Medical Center, New York
University of Rochester Medicine, Rochester
North CarolinaUniversity of North Carolina at Chapel Hill, Chapel Hill
Duke University School of Medicine, Durham
Pulmonix, LLC, Greensboro
East Carolina University Health, Greenville
Wakemed Health and Hospital, Raleigh
OhioUniversity of Cincinnati Medical Center, Cincinnati
The Cleveland Clinic, Cleveland
University Hospitals Cleveland Medical Center, Cleveland
Ohio State Richard M. Ross Heart Hospital, Columbus
OklahomaThe University of Oklahoma Health Sciences Center, Oklahoma City
Oklahoma Heart Institute, Tulsa
OregonSummit Health, Bend
Legacy Research Institute, Portland
Oregon Health & Science University, Portland
PennsylvaniaSt. Luke's University Health Network, Bethlehem
Temple Lung Center, Philadelphia
Temple University Hospital, Philadelphia
Thomas Jefferson University - Sidney Kimmel Medical College, Philadelphia
University of Pennsylvania, Philadelphia
South CarolinaAnMed Health, Anderson
Medical University of South Carolina, Charleston
TennesseeStatCare Pulmonary Consultants and Center for Biomedical Research, Knoxville
TexasBaylor University Medical Center, Dallas
UT Southwest Medical Center Heart and Lung Clinic, Dallas
Houston Methodist, Houston
University of Texas Health Science Center at Houston, Houston
UtahIntermountain Medical Center, Murray
University of Utah Health, Salt Lake City
VirginiaInova Medical Group, Falls Church
Sentara Norfolk General Hospital, Norfolk
Pulmonary Associates of Richmond, Inc., Richmond
Virginia Commonwealth University, Richmond
West VirginiaMarshall Health, Huntington
WisconsinUniversity of Wisconsin Hospital and Clinics, Madison
Aurora St. Luke's Medical Center, Milwaukee
Medical College of Wisconsin, Milwaukee

Conditions

From ClinicalTrials.gov, data retrieved Sep 30, 2026. Each study sets its own eligibility; the study team decides who can join.