Ulrich, S;  Department of Pulmonology, University Hospital Zurich, Zurich,
Milger, K;Halank, M;et al

Respiration; international review of thoracic diseases,2026 Oct 17

• 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.