Prognostic factors in interstitial lung disease-associated pulmonary hypertension: data from the HYPID cohort and the French Pulmonary Hypertension Registry - PubMed
3 hours ago
- #pulmonary hypertension
- #interstitial lung disease
- #prognostic factors
- Pulmonary hypertension (PH) frequently complicates interstitial lung diseases (ILD), worsening outcomes.
- The study analyzed 581 ILD-PH patients from the HYPID cohort and French Registry (2007-2022).
- Common ILD diagnoses included combined pulmonary fibrosis and emphysema syndrome (30.8%) and idiopathic pulmonary fibrosis (29.6%).
- Median transplant-free survival was 17 months; 37% of patients received off-label PH therapy.
- Independent predictors of 1-year mortality/transplant were male sex, WHO functional class III/IV, 6 MWD ≤228 m, PVR >5 Wood units, and no PH therapy.
- Non-invasive (6 MWD, WHO FC) and invasive (PVR) variables are prognostic; PH medication may improve outcomes.