• Carmine Dario Vizza, Marius M Hoeper, Doerte Huscher, David Pittrow, Nicola Benjamin, Karen M Olsson, H Ardeschir Ghofrani, Matthias Held, Hans Klose, Tobias Lange, Stephan Rosenkranz, Daniel Dumitrescu, Roberto Badagliacca, Martin Claussen, Michael Halank, Anton Vonk-Noordegraaf, Dirk Skowasch, Ralf Ewert, GibbsJ Simon RJSRNational Heart and Lung Institute, Imperial College London, London, United Kingdom., Marion Delcroix, Andris Skride, Gerry Coghlan, Silvia Ulrich, Christian Opitz, Harald Kaemmerer, Oliver Distler, and Ekkehard Grünig.
• Pulmonary Hypertension Unit, Department of Cardiovascular and Respiratory Diseases, Sapienza University of Rome, Rome, Italy. Electronic address: dario.vizza@uniroma1.it.
• Chest. 2021 Aug 1; 160 (2): 678-689.

BackgroundPulmonary hypertension (PH) in COPD is a poorly investigated clinical condition.Research QuestionWhich factors determine the outcome of PH in COPD?Study Design And MethodsWe analyzed the characteristics and outcome of patients enrolled in the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA) with moderate or severe PH in COPD as defined during the 6th PH World Symposium who received medical therapy for PH and compared them with patients with idiopathic pulmonary arterial hypertension (IPAH).ResultsThe population included incident patients with moderate PH in COPD (n = 68), with severe PH in COPD (n = 307), and with IPAH (n = 489). Patients with PH in COPD were older, predominantly male, and treated mainly with phosphodiesterase-5 inhibitors. Despite similar hemodynamic impairment, patients with PH in COPD achieved a worse 6-min walking distance (6MWD) and showed a more advanced World Health Organization functional class (WHO FC). Transplant-free survival rates at 1, 3, and 5 years were higher in the IPAH group than in the PH in COPD group (IPAH: 94%, 75%, and 55% vs PH in COPD: 86%, 55%, and 38%; P = .004). Risk factors for poor outcomes in PH in COPD were male sex, low 6MWD, and high pulmonary vascular resistance (PVR). In patients with severe PH in COPD, improvements in 6MWD by ≥ 30 m or improvements in WHO FC after initiation of medical therapy were associated with better outcomes.InterpretationPatients with PH in COPD were functionally more impaired and had a poorer outcome than patients with IPAH. Predictors of death in the PH in COPD group were sex, 6MWD, and PVR. Our data raise the hypothesis that some patients with severe PH in COPD may benefit from PH treatment. Randomized controlled studies are necessary to explore this hypothesis further.Trial RegistryClinicalTrials.gov; No.: NCT01347216; URL: www.clinicaltrials.gov.Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.

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