Pulmonary hypertension associated with lung disease - an expert review from the Cologne Conference

Pulmonary hypertension associated with chronic lung disease (PH_lung) is a frequent complication of chronic obstructive pulmonary disease, 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. Following the 7th World Symposium on Pulmonary Hypertension, a panel of experts reviewed from Germany and International collaborators summarized recent evidence and discussed unresolved issues relevant to everyday clinical practice. 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 pulmonary hypertension associated with interstitial lung disease in several countries, no pulmonary hypertension-specific therapy is currently recommended routinely for COPD-associated PH, highlighting the need for careful patient phenotyping and individualized management in expert centres. The review further addresses indications for lung transplantation and outlines future research priorities. Earlier recognition, multidisciplinary collaboration, and continued translational and clinical research are essential to improve outcomes in this highly vulnerable patient population.