Exercise Hemodynamics in Patients With Pulmonary Fibrosis

Study Overview
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Brief Summary
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The study evaluates the rest and exercise hemodynamics of patients presenting either idiopathic fibrotic pulmonary disease or pulmonary fibrosis secondary to connective tissue disease.

Detailed Description
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Patients with fibrotic pulmonary disease of the above origin will undergo detailed assessment of functional status with the following examinations: pulmonary function tests, echocardiogram, 6 minute exercise test, cardiopulmonary exercise test (in the absence of contra-indications) and finally right heart catheterization. Hemodynamic assessment will take place at rest and at exercise following the exercise protocol that was recommended by Herve et al (ERJ 2015) using a cycle ergometer at bedside. The purposes of the study are: to evaluate the response of the hemodynamic parameters at exercise compared to the resting parameters in this patient group. to discriminate between precapillary from postcapillary etiology of pulmonary hypertension. to correlate non-invasive parameters of functional limitation with invasive hemodynamic parameters. to assess the prognostic role of exercise induced pulmonary hypertension in the specific patient group.

Official Title
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Hemodynamic Study of the Pulmonary Circulation in Exercise in Patients With Pulmonary Fibrosis

Conditions
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Exercise Pulmonary Hypertension

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: diagnosis of pulmonary fibrosis secondary to connective tissue disease, according to the American College of Rheumatology/European League Against Rheumatism (ACR-EULAR) revised classification criteria , or diagnosis of idiopathic fibrotic diffuse pulmonary disease according to American Thoracic Society/European Respiratory Society (ATS/ERS) guidelines Forced vital capacity (FVC) ≥ 40 % Exclusion Criteria: Presence of contra-indications to exercise tests, such as acute myocardial infarction, unstable angina, uncontrollable arrythmia or skeletal disease that limits the ability to exercise. History of pulmonary embolism during the year before enrollment Presence of severe valvular disease. Current treatment with vasoactive drugs of the pulmonary circulation Pulmonary wedge pressure (PAWP) at rest > 15 mmHg

Ages Eligible for Study
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Minimum Age: 20 Years
Maximum Age: 75 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| normal rest and exercise hemodynamics<br> | |
| normal rest and abnormal exercise hemodynamics<br> | |
| resting pulmonary hypertension<br> | |
| abnormal wedge pressure at exercise<br> | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Change in total pulmonary resistance (TPR) from rest to exercise | The patient undergoes right heart catheterization and all hemodynamic variables are measured at rest and at exercise. TPR is derived by the following equation : mean pulmonary artery pressure/ cardiac output (mPAP/CO). TPR is measured at rest and at exercise following a specific protocol. The measurements are made at rest, then at the sequential exercise steps of increasing work resistance. | 2nd minute,4th minute, 6th minute, 8th minute |