Pulmonary Hypertension Screening in Patients With Interstitial Lung Disease for Earlier Detection

Study Overview
=================
Brief Summary
-----------------
Study GMS-PH-001 is a multicenter, open-label, non-randomized study to prospectively evaluate screening strategies of pulmonary hypertension (PH) in patients with interstitial lung disease (ILD).

Detailed Description
-----------------
In this study, subjects will undergo a broad range of clinical assessments that are potentially associated with PH. Study data will be used to identify and weigh specific clinical parameters based on their prognostic significance for right heart catheterization (RHC)-confirmed PH. There is no study drug under investigation in this study. The study consists of 2 study visits: a Screening Visit and Study Visit 1. Clinical assessments include pulmonary function tests (PFTs); high resolution computed tomography (HRCT); physical examination; 6-Minute Walk Test; blood draw for clinical laboratory parameters, plasma brain natriuretic peptide (BNP) concentration and plasma N-terminal pro-BNP (NT-proBNP) concentration; echocardiography; University of California San Diego Shortness of Breath Questionnaire (UCSD SOBQ); King's Brief Interstitial Lung Disease Questionnaire (K-BILD); 36-Item Short Form Survey (SF-36); Pulmonary Hypertension Functional Classification Self Report (PH-FC-SR); Investigator's Suspicion of PH Questionnaire; adverse event (AE) monitoring; and RHC. As the primary objective of this study is to collect a broad range of clinical parameters in patients with ILD, it is expected that numerous exploratory and post-hoc analyses will be performed to identify and weigh specific parameters based on their prognostic significance for PH in this patient population with the goal of developing a screening algorithm for PH in patients with ILD.

Official Title
-----------------
Pulmonary Hypertension Screening in Patients With Interstitial Lung Disease for Earlier Detection

Conditions
-----------------
Interstitial Lung Disease, Pulmonary Hypertension

Intervention / Treatment
-----------------
* Procedure: Right heart catheterization (RHC)


Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria Patient gives voluntary written informed consent to participate in the study. Patients with a diagnosis of ILD based on computed tomography imaging, including: Idiopathic interstitial pneumonia, including idiopathic pulmonary fibrosis Connective tissue disease-associated ILD with forced vital capacity (FVC) <70% Hypersensitivity pneumonitis Scleroderma-related ILD Autoimmune ILD Nonspecific interstitial pneumonia Occupational lung disease Combined pulmonary fibrosis and emphysema Patients must have at least 2 signs or symptoms suggestive of PH, as specified by the study protocol. Exclusion Criteria Prior RHC with mPAP >20 mmHg. Currently on a Food and Drug Administration (FDA)-approved pulmonary arterial hypertension medication. Diagnosed with chronic obstructive pulmonary disease. Uncontrolled or untreated sleep apnea. Pulmonary embolism within the past 3 months. History of ischemic heart disease or left-sided myocardial dysfunction within 12 months of Screening, defined as left ventricular ejection fraction <40% or pulmonary capillary wedge pressure >15 mmHg. Any other clinical features that, in the opinion of the Investigator, might adversely affect interpretation of study data or study safety, or make the patient unsuitable for RHC.

Ages Eligible for Study
-----------------
Minimum Age: 18 Years

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
No

Study Plan
=================
How is the study designed?
-----------------

Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental study arm<br>All enrolled patients in this single arm study will receive a RHC. | Procedure: Right heart catheterization (RHC)<br>* RHC to evaluate pulmonary hemodynamics<br>|

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Percentage of patients with PH as indicated by RHC | Mean pulmonary artery pressure (mPAP) >20 mmHg with pulmonary artery wedge pressure (PAWP) ≤15 mmHg and pulmonary vascular resistance (PVR) ≥2 WU. | Through study completion, approximately 3 weeks. | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Percentage of patients with severe PH as indicated by RHC | mPAP >20 mmHg with PAWP ≤15 mmHg and PVR >5 WU. | Through study completion, approximately 3 weeks. | 

 Terms related to the study
=================
Keywords Provided by Centre Hospitalier Valida
-----------------
ILD, PH, PH-ILD