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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).
The purpose of this study is to capture and analyze patient characteristics, treatment patterns, and clinical outcomes of patients with PH-ILD, with the goal of enhancing our current understanding of the disease state and its treatment outcomes. The study also aims to describe the disease trajectory of ILD patients at risk of pulmonary hypertension (PH).
In this registry, patients will be enrolled into 1 of 4 cohorts. Cohort 1 will include approximately 200 patients who are not receiving inhaled treprostinil at time of study enrollment. Cohort 2 will include approximately 300 patients who are newly initiated on Tyvaso/Tyvaso DPI. Cohort 3 will include approximately 300 patients who have been receiving Tyvaso/Tyvaso DPI for >90 days. Cohort 4 will include approximately 100 ILD patients with a prior right heart catheterization (RHC) not meeting the definition of PH.
As part of the registry, assessments will be completed for data collection in 6- or 12 month intervals, unless the data are already available via standard of care. This patient registry will follow patients for up to 5 years after enrollment.
Assessments include pulmonary function tests (PFTs) including diffusing capacity of the lungs for carbon monoxide (DLCO); high resolution computed tomography (HRCT); vital signs; 6-Minute Walk Test; blood draw for plasma N-terminal pro-BNP (NT-proBNP) concentration, genetics analysis, biomarker analysis; echocardiography; EuroQoL 5 Dimension 5 Level Questionnaire (EQ-5D-5L); King's Brief Interstitial Lung Disease Questionnaire (K-BILD); Living with Pulmonary Fibrosis Questionnaire (L-PF); Therapy Administration Questionnaire; emPHasis-10 Questionnaire; healthcare resource utilization; current medications and rehabilitation; WHO functional class; transplant status; palliative care data; survival data; and RHC.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Cohort 1 | Cohort 1 will include approximately 200 patients who are not receiving inhaled treprostinil at time of study enrollment. |
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| Cohort 2 | Cohort 2 will include approximately 300 patients who are newly initiated on Tyvaso/Tyvaso DPI. For patients to be eligible for Cohort 2, they must have initiated Tyvaso/Tyvaso DPI at Baseline or ≤ 90 days prior to Baseline. |
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| Cohort 3 | Cohort 3 will include approximately 300 patients who have been receiving Tyvaso/Tyvaso DPI for > 90 days prior to Baseline. |
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| Cohort 4 | Cohort 4 will include approximately 100 ILD patients with a prior RHC not meeting the definition of PH but has a pulmonary artery wedge pressure ≤ 15 mmHg. |
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Prospective study assessments | Other | Patients will complete prospective assessments at 6- and 12-month intervals for up to 5 years. There will be 1 check in at the first 3 months of enrollment. Follow up visits will include a ±45 day visit window. If an assessment is performed within 45 days of the visit window as part of the patient's standard of care, those values will be used in place of a prospective assessment. |
| Measure | Description | Time Frame |
|---|---|---|
| Baseline characteristics as described by demographics, medical history, ILD history, PH history, family history (comorbidities), and diagnostic right heart catheterization values. | Baseline |
| Measure | Description | Time Frame |
|---|---|---|
| Changes in clinical features relating to patients' interstitial lung disease and pulmonary hypertension | Followed prospectively for a maximum of 5 years from date of enrollment into the registry |
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Inclusion Criteria:
Adults aged 18 years or older
Diagnosis of fibrotic ILD based on high-resolution computed tomography imaging, including but not limited to:
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).
For patients to be eligible for Cohort 1, they must not be receiving inhaled treprostinil at Baseline.
For patients to be eligible for Cohort 2, they must have initiated
Tyvaso/Tyvaso DPI at 1 of the following time points:
Exclusion Criteria:
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.
Confirmed diagnosis of Group 3 PH associated with lung diseases and or hypoxia other than fibrotic ILD, as outlined in Inclusion Criterion 2.
Patients receiving inhaled treprostinil (Yutrepiaâ„¢) at Baseline. Patients receiving other forms of PH therapy will be eligible for enrollment.
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Patients with a diagnosis of pulmonary hypertension associated with interstitial lung disease or interstitial lung disease. This includes patients who are and aren't receiving inhaled treprostinil, excluding patients receiving Yutrepiaâ„¢ at Baseline.
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| United Therapeutics Global Medical Information | Contact | 919-485-8350 | clinicaltrials@unither.com |
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| University of Alabama at Birmingham | Recruiting | Birmingham | Alabama | 35294 | United States |
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For patients consenting to genomic analysis, a blood sample will be collected. These samples will be shipped to the central laboratory for processing and storage prior to analysis. Genomic analysis will be analyzed for genetic markers that may be associated with clinical response and tolerability.
For patients consenting to optional biomarker sample, blood will be collected for the evaluation of biomarkers (specific targets to be determined).
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| Banner Health - University Medical Center Phoenix | Recruiting | Phoenix | Arizona | 85006 | United States |
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| Cedars-Sinai Medical Center | Recruiting | Los Angeles | California | 90048 | United States |
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| University of Colorado - Anschutz Medical Campus | Recruiting | Aurora | Colorado | 80045 | United States |
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| National Jewish Health | Recruiting | Denver | Colorado | 80206 | United States |
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| Pasquerilla Healthcare Center - Georgetown University | Recruiting | Washington D.C. | District of Columbia | 20007 | United States |
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| University of Florida | Recruiting | Gainesville | Florida | 32610 | United States |
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| NCH Research Institute | Recruiting | Naples | Florida | 34102 | United States |
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| Advent Health Medical Group Advanced Lung Disease | Recruiting | Orlando | Florida | 32804 | United States |
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| Intercoastal Medical Group | Recruiting | Sarasota | Florida | 34239 | United States |
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| Tampa General Hospital/University of South Florida Health | Recruiting | Tampa | Florida | 33606 | United States |
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| Weston Hospital - Cleveland Clinic Florida | Recruiting | Weston | Florida | 33331 | United States |
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| Emory University | Recruiting | Atlanta | Georgia | 30322 | United States |
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| Northwestern University | Recruiting | Chicago | Illinois | 60611 | United States |
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| Loyola University Medical Center | Recruiting | Maywood | Illinois | 60153 | United States |
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| Advocate Christ Medical Center | Recruiting | Oak Lawn | Illinois | 60453 | United States |
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| Ascension St. Vincent | Recruiting | Indianapolis | Indiana | 46260 | United States |
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| University of Kansas Medical Center | Recruiting | Kansas City | Kansas | 66160 | United States |
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| Norton Pulmonary Specialists | Recruiting | Louisville | Kentucky | 40202 | United States |
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| University Medical Center - New Orleans | Recruiting | New Orleans | Louisiana | 70112 | United States |
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| University of Maryland Medical Center | Recruiting | Baltimore | Maryland | 21201 | United States |
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| Washington University School of Medicine | Recruiting | St Louis | Missouri | 63110 | United States |
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| Dartmouth Hitchcock Medical Center | Recruiting | Lebanon | New Hampshire | 03766 | United States |
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| Morristown Medical Center | Recruiting | Morristown | New Jersey | 07960 | United States |
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| HMH Jersey Shore University Medical Ctr. | Recruiting | Neptune City | New Jersey | 07753 | United States |
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| University of New Mexico Health Sciences Center | Recruiting | Albuquerque | New Mexico | 87131 | United States |
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| Northwell Health | Recruiting | New Hyde Park | New York | 11042 | United States |
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| New York University Langone Medical Center | Recruiting | New York | New York | 10016 | United States |
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| New York Presbyterian Hospital - Weill Cornell Medicine | Recruiting | New York | New York | 10021 | United States |
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| University of Rochester Medicine | Recruiting | Rochester | New York | 14623 | United States |
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| University of North Carolina at Chapel Hill | Recruiting | Chapel Hill | North Carolina | 27514 | United States |
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| Duke University School of Medicine | Recruiting | Durham | North Carolina | 27710 | United States |
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| Pulmonix, LLC | Recruiting | Greensboro | North Carolina | 27403 | United States |
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| East Carolina University Health | Recruiting | Greenville | North Carolina | 27834 | United States |
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| Wakemed Health and Hospital | Recruiting | Raleigh | North Carolina | 27610-1231 | United States |
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| University of Cincinnati Medical Center | Recruiting | Cincinnati | Ohio | 45219 | United States |
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| University Hospitals Cleveland Medical Center | Recruiting | Cleveland | Ohio | 44106 | United States |
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| The Cleveland Clinic | Recruiting | Cleveland | Ohio | 44195 | United States |
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| Ohio State Richard M. Ross Heart Hospital | Recruiting | Columbus | Ohio | 43210 | United States |
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| The University of Oklahoma Health Sciences Center | Recruiting | Oklahoma City | Oklahoma | 73104 | United States |
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| Oklahoma Heart Institute | Recruiting | Tulsa | Oklahoma | 74104 | United States |
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| Summit Health | Recruiting | Bend | Oregon | 97701 | United States |
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| Legacy Research Institute | Recruiting | Portland | Oregon | 97210 | United States |
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| Oregon Health & Science University | Recruiting | Portland | Oregon | 97239 | United States |
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| St. Luke's University Health Network | Recruiting | Bethlehem | Pennsylvania | 18015 | United States |
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| University of Pennsylvania | Recruiting | Philadelphia | Pennsylvania | 19104 | United States |
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| Thomas Jefferson University - Sidney Kimmel Medical College | Recruiting | Philadelphia | Pennsylvania | 19107 | United States |
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| Temple Lung Center | Recruiting | Philadelphia | Pennsylvania | 19140 | United States |
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| Temple University Hospital | Recruiting | Philadelphia | Pennsylvania | 19140 | United States |
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| AnMed Health | Recruiting | Anderson | South Carolina | 29621 | United States |
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| Medical University of South Carolina | Recruiting | Charleston | South Carolina | 29425 | United States |
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| StatCare Pulmonary Consultants and Center for Biomedical Research | Recruiting | Knoxville | Tennessee | 37909 | United States |
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| Baylor University Medical Center | Recruiting | Dallas | Texas | 75246 | United States |
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| UT Southwest Medical Center Heart and Lung Clinic | Recruiting | Dallas | Texas | 75390 | United States |
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| Houston Methodist | Recruiting | Houston | Texas | 77030 | United States |
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| University of Texas Health Science Center at Houston | Recruiting | Houston | Texas | 77030 | United States |
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| Intermountain Medical Center | Recruiting | Murray | Utah | 84157 | United States |
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| University of Utah Health | Recruiting | Salt Lake City | Utah | 84108 | United States |
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| Inova Medical Group | Recruiting | Falls Church | Virginia | 22042 | United States |
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| Sentara Norfolk General Hospital | Recruiting | Norfolk | Virginia | 23507 | United States |
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| Pulmonary Associates of Richmond, Inc. | Recruiting | Richmond | Virginia | 23230 | United States |
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| Virginia Commonwealth University | Recruiting | Richmond | Virginia | 23298 | United States |
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| Marshall Health | Recruiting | Huntington | West Virginia | 25701 | United States |
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| University of Wisconsin Hospital and Clinics | Recruiting | Madison | Wisconsin | 53792 | United States |
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| Aurora St. Luke's Medical Center | Recruiting | Milwaukee | Wisconsin | 53215 | United States |
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| Medical College of Wisconsin | Recruiting | Milwaukee | Wisconsin | 53226 | United States |
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| Auxilio Mutuo Hospital | Recruiting | Guaynabo | Puerto Rico | 00968 | Puerto Rico |
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| ID | Term |
|---|---|
| D000860 | Hypoxia |
| D006976 | Hypertension, Pulmonary |
| D017563 | Lung Diseases, Interstitial |
| ID | Term |
|---|---|
| D012818 | Signs and Symptoms, Respiratory |
| D012816 | Signs and Symptoms |
| D013568 | Pathological Conditions, Signs and Symptoms |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
| D006973 | Hypertension |
| D014652 | Vascular Diseases |
| D002318 | Cardiovascular Diseases |
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