Pulmonary Hypertension Associated With Lung Disease: An Expert Review From the Cologne Conference
12 affiliations
- 1Department of Pulmonology, University and University Hospital Zurich, Zurich, Switzerland
- 2Department of Pulmonology, University of Graz, Graz, Austria
- 3Department of Pulmonology, University of Dresden, Dresden, Germany
- 4Department of Pulmonology, University of Hamburg, Hamburg, Germany
- 5Internal Medicine II, Respiratory Medicine and Respiratory Care, Bad Reichenhall, Germany
- 6Internal Medicine, Pulmonology and Cardiology, Neuwittelsbach Hospital, Muenchen, Germany
- 7Department of Pulmonary Medicine, Pulmonary Hypertension and Interstitial Lung Disease, Amsterdam, The Netherlands
- 8Department of Pulmonology, Internal Medicine, Neurology and Dermatology, University Hospital Leipzig, Leipzig, Germany
- 9Pulmonary Hypertension Clinic, University of Virginia School of Medicine, Charlottesville, VA, United States
- 10Medical Clinic and Outpatient Clinic II, Heart Centre, University Hospital Bonn, Bonn, Germany
- 11Department of Respiratory Medicine and Internal Medicine Intensive Care, University Clinic Aachen, Aachen, Germany
- 12Internal and Respiratory Medicine, Ludwig Maximilian University of Munich, Munich, Germany
Abstract
Background: Pulmonary hypertension (PH) associated with chronic lung disease (CLD) (PH_lung) is a frequent complication of chronic obstructive pulmonary disease (COPD), interstitial lung disease, and other parenchymal lung disorders, and is associated with impaired exercise capacity, reduced quality of life and poor survival. Despite increasing recognition of its clinical importance, major challenges remain regarding early diagnosis, hemodynamic classification, and optimal treatment.
Summary: This review summarizes current concepts of the diagnosis and management of PH_lung, with particular emphasis on multimodal screening strategies integrating pulmonary function testing, biomarkers, echocardiography, cardiopulmonary exercise testing, and thoracic imaging to improve patient selection for right heart catheterization. We discuss practical aspects of hemodynamic assessment, overlapping phenotypes, including connective tissue disease, sarcoidosis, and the emerging pulmonary vascular “lung phenotype,” and current evidence supporting targeted therapies with emphasis on patients’ pathways in real-world settings. While inhaled treprostinil has become the first approved treatment for PH associated with interstitial lung disease in some countries, no PH-specific therapy is currently recommended routinely for COPD-associated PH, highlighting the need for careful patient phenotyping and individualized management in expert centers. Earlier recognition, multidisciplinary collaboration, and continued translational and clinical research are essential to improve outcomes in this highly vulnerable patient population.
Key Messages: Patients with PH_lung may present in pulmonary clinics with worsening CLD or are diagnosed in the work-up of PH. PH_lung is a severe condition that should be managed in expert centers with the possibility of advanced therapies, including transplantation and inclusion in clinical trials.
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BibTeX
@article{ulrich2026pulmonary,
title = {{Pulmonary Hypertension Associated With Lung Disease: An Expert Review From the Cologne Conference}},
author = {Silvia Ulrich and Katrin Milger and Michael Halank and Hans Klose and Tobias J. Lange and Hanno H. Leuchte and Esther J. Nossent and Hans-Jürgen Seyfarth and Oksana A. Shlobin and Dirk Skowasch and Michael Dreher and Juergen Behr},
journal = {Respiration},
year = {2026},
month = oct,
doi = {10.1159/res/aepag019},
url = {https://doi.org/10.1159/res/aepag019},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Pulmonary Hypertension Associated With Lung Disease: An Expert Review From the Cologne Conference
AU - Silvia Ulrich
AU - Katrin Milger
AU - Michael Halank
AU - Hans Klose
AU - Tobias J. Lange
AU - Hanno H. Leuchte
AU - Esther J. Nossent
AU - Hans-Jürgen Seyfarth
AU - Oksana A. Shlobin
AU - Dirk Skowasch
AU - Michael Dreher
AU - Juergen Behr
PY - 2026
DA - 2026/10/17
JO - Respiration
DO - 10.1159/res/aepag019
UR - https://doi.org/10.1159/res/aepag019
AB - Background: Pulmonary hypertension (PH) associated with chronic lung disease (CLD) (PH_lung) is a frequent complication of chronic obstructive pulmonary disease (COPD), interstitial lung disease, and other parenchymal lung disorders, and is associated with impaired exercise capacity, reduced quality of life and poor survival. Despite increasing recognition of its clinical importance, major challenges remain regarding early diagnosis, hemodynamic classification, and optimal treatment. Summary: This review summarizes current concepts of the diagnosis and management of PH_lung, with particular emphasis on multimodal screening strategies integrating pulmonary function testing, biomarkers, echocardiography, cardiopulmonary exercise testing, and thoracic imaging to improve patient selection for right heart catheterization. We discuss practical aspects of hemodynamic assessment, overlapping phenotypes, including connective tissue disease, sarcoidosis, and the emerging pulmonary vascular “lung phenotype,” and current evidence supporting targeted therapies with emphasis on patients’ pathways in real-world settings. While inhaled treprostinil has become the first approved treatment for PH associated with interstitial lung disease in some countries, no PH-specific therapy is currently recommended routinely for COPD-associated PH, highlighting the need for careful patient phenotyping and individualized management in expert centers. Earlier recognition, multidisciplinary collaboration, and continued translational and clinical research are essential to improve outcomes in this highly vulnerable patient population. Key Messages: Patients with PH_lung may present in pulmonary clinics with worsening CLD or are diagnosed in the work-up of PH. PH_lung is a severe condition that should be managed in expert centers with the possibility of advanced therapies, including transplantation and inclusion in clinical trials.
SN - 0025-7931
SN - 1423-0356
ER -