On Monday, 28 September 2025, a State of the Art Session was held at the European Respiratory Society (ERS) Annual Congress 2025 titled: “Pulmonary Hypertension associated with lung diseases: the most burning questions.” The session was chaired by Prof. Dr. Olivier Sitbon, Prof. Dr. David Montani, and Dr. Nesrin Ocal. Leading experts shared the latest insights into the diagnosis, management, and emerging treatment approaches for pulmonary hypertension in the context of chronic lung diseases.
What follows is a short summary by Louise Bouman, Chair of the Dutch Pulmonary Hypertension Association and Member of the Board of the Alliance for Pulmonary Hypertension.
David Kiely: Diagnosis of pulmonary hypertension associated with lung diseases: which patients should undergo Right Heart Catheterization (RHC)?
- Pulmonary hypertension is common in people with chronic lung disease and can
worsen symptoms and reduce life expectancy. - If pulmonary hypertension is suspected, an echocardiogram is usually performed. Results are considered together with other tests such as lung function and Computerised Tomography scans.
- If the diagnosis of pulmonary hypertension will guide treatment decisions, a right heart catheterization may be needed. This is done in specialized centers in stable patients and provides a full assessment of blood flow in the lungs.
- Diagnosing pulmonary hypertension in lung disease is challenging. There are few reliable non-invasive tests, limited access to right heart catheterization, and no fully integrated care pathways.
- Research is needed to develop accessible, reliable non-invasive tests to detect pulmonary hypertension early and safely in lung disease.
Lucilla Piccari: pulmonary arterial hypertension drugs in the management of pulmonary hypertension associated with lung diseases: clinical trials and clinical practice
- There is some retrospective evidence supporting the use of pulmonary arterial hypertension drugs in WHO Group 3 (pulmonary hypertension caused by lung disease), but results from clinical trials (Randomised Controlled Trials, RCTs) have been disappointing.
- It is important to treat patients with hemodynamically confirmed pulmonary hypertension (proven by right heart catheterization).
- There is still work to do to identify meaningful outcomes for patients with WHO Group 3 pulmonary hypertension guidelines.
- Interstitial lung disease (ILD) pulmonary hypertension: Treat if pulmonary vascular resistance (PVR) ≥ 4 Wood units; do not treat if pulmonary vascular resistance < 3 Wood Units; 3–4 Wood Units is a “grey zone.”
- Chronic Obstructive Pulmonary Disease (COPD) pulmonary hypertension: treatment is decided case by case, usually starting with PDE5 inhibitors.
- In clinical practice, pulmonary arterial hypertension drugs are used fairly widely and generally according to guidelines.
Martin Kolb: Novel possible treatment approaches for pulmonary arterial hypertension associated with lung diseases: a molecular perspective:
- In idiopathic pulmonary fibrosis (IPF), the blood vessels in the lungs play an important role in disease progression.
- In fibrotic lungs, the pulmonary blood vessels undergo abnormal changes (remodeling).
- This involves dysfunction of several cell types in the blood vessels, including smooth muscle cells, endothelial cells, basement membranes, and mesenchymal cells.
- These remodeled blood vessels can send signals that worsen fibrosis, making the lung scarring progress further.
Marius Hoeper: Lung phenotype in pulmonary arterial hypertension
Idiopathic pulmonary arterial hypertension with a lung phenotype…….is not pulmonary
arterial hypertension. Some patients diagnosed with idiopathic pulmonary arterial hypertension actually have a lung-related form of pulmonary hypertension, similar to WHO Group 3 pulmonary hypertension.
Key features include:
- Low lung diffusion capacity <45% (DLCO)
- Low oxygen levels (hypoxemia)
- History of heavy smoking
- Imaging and tissue studies show loss of small blood vessels and changes in lung tissue resembling combined pulmonary fibrosis and emphysema.
- These patients may need to be reclassified and treated as WHO Group 3 pulmonary hypertension rather than idiopathic pulmonary hypertension.