Management of Pulmonary Hypertension associated with Chronic Lung Disease: Results of the ERS Clinical Research Collaboration PHAROS survey

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Introduction: Pulmonary hypertension associated with chronic lung diseases (PH-CLD) is an heterogenous group in terms of clinical presentation and management. Pooling data from registries could help to delineate different phenotypes. This survey aimed to evaluate the management of PH-CLD across different European PH centers and to assess the feasibility of data registry integration. Methodology: The study was initiated within the frameworks of ERS Clinical Research Collaboration PHAROS and European Reference Network for rare lung diseases (ERN-Lung). A survey was proposed, focusing on patient volume, diagnostic, treatment, registry inclusion and patient categorization. Results: Of 34 participating European PH centers (91% ERN-Lung or designated national centers), 88% mentioned managing PH-CLD patients (average 75, range: 10–293), 82% using right heart catheterization for diagnosing PH-CLD, mainly in case of high echocardiographic suspicion, 85% enrolling PH-CLD patients in registries. Interstitial lung disease was the most prevalent underlying condition. An average of 26 (0–150) PH-CLD patients/center were treated with PAH-approved drugs. There was an important heterogeneity in patient categorization, mainly for PAH with low DLCO, and in data collection. Morbidity and mortality were the preferred endpoints for future trials in PH-CLD. Conclusion: This survey provides insights into PH-CLD management across Europe. Significant disparities in patient categorization, data collection, diagnosis and treatment preclude realistic data integration. There is a critical need for a new prospective registry to accurately define specific PH-CLD phenotypes.

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