Pulmonary Hypertension in Interstitial Lung Disease

Observational Study of Patients With Pre Capillary Pulmonary Hypertension

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.

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.

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