Pulmonary Hypertension in Interstitial Lung Disease

Study Overview
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Brief Summary
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HYPID study is an observational and prospective study of patients with interstitial lung disease and pre capillary hypertension diagnosed by right heart sided catheterization. The primary aim of the study is to identify prognostic factors.

Detailed Description
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Pre capillary pulmonary hypertension (PH) may be present in patients with diffuse interstitial lung disease. In this context, PH represents an important factor of morbidity and mortality for these patients. One of the purposes of HYPID is to determine predictive factors of mortality within this cohort of patients. In order to reach that aim,the study includes an evaluation based on exams conducted for the routine follow-up of patients. Each patient will be followed during 2 years at least.

Official Title
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Observational Study of Patients With Pre Capillary Pulmonary Hypertension

Conditions
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Pulmonary Arterial Hypertension, Diffuse Interstitial Lung Disease

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: pre capillary pulmonary hypertension at right heart sided catheterization with: mPAP > or = 25 mmHg,PCWP < or = 15 mmHg interstitial lung disease with diffuse infiltrative opacities on chest CT scan Exclusion Criteria: pulmonary hypertension related to a thromboembolic disease respiratory disease other than diffuse interstitial lung disease any etiological factor of pulmonary arterial hypertension based on DANA POINT classification other than diffuse interstitial lung disease any progressive disease associated to a life expectancy less than 6 months other than pulmonary hypertension, diffuse interstitial lung disease and respiratory insufficiency

Ages Eligible for Study
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Minimum Age: 18 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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What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Overall survival | Determine predictive factors of mortality | 2 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Progression-free survival (1) | Determine cause of death | 2 years | 
| Progression-free survival (2) | Determine survival and time to worsening | 2 years | 
| Progression-free survival (3) | Analyse clinical, functional respiratory, hemodynamic features and changing characteristics of pulmonary hypertension | 2 years | 
| Progression-free survival (4) | Document the efficacy of PH specific treatment | 2 years | 
| Progression-free survival (5) | Evaluate the level of pulmonary arterial hypertension and compare the functional characteristics of patients with moderate or severe pulmonary hypertension | 2 years | 
| Progression-free survival (6) | Analyse clinical, functional respiratory, hemodynamic features and changing characteristics of pulmonary hypertension | 2 years | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Pulmonary arterial hypertension,, Interstitial lung disease at HRCT