Carmine Dario Vizza1, Marius M Hoeper2, Doerte Huscher3, David Pittrow4, Nicola Benjamin5, Karen M Olsson6, H Ardeschir Ghofrani7, Matthias Held8, Hans Klose9, Tobias Lange10, Stephan Rosenkranz11, Daniel Dumitrescu12, Roberto Badagliacca13, Martin Claussen14, Michael Halank15, Anton Vonk-Noordegraaf16, Dirk Skowasch17, Ralf Ewert18, J Simon R Gibbs19, Marion Delcroix20, Andris Skride21, Gerry Coghlan22, Silvia Ulrich23, Christian Opitz24, Harald Kaemmerer25, Oliver Distler26, Ekkehard Grünig5. 1. Pulmonary Hypertension Unit, Department of Cardiovascular and Respiratory Diseases, Sapienza University of Rome, Rome, Italy. Electronic address: dario.vizza@uniroma1.it. 2. Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany; German Center of Lung Research (DZL), Germany. 3. Institute of Biometry and Clinical Epidemiology and Berlin Institute of Health, Charité-Universitaetsmedizin, Berlin, Germany. 4. Institute for Clinical Pharmacology, Medical Faculty, Technical University, Dresden, Germany. 5. German Center of Lung Research (DZL), Germany; Centre for Pulmonary Hypertension, Thoraxklinik Heidelberg gGmbH at Heidelberg University, Hospital, Heidelberg, Germany. 6. German Center of Lung Research (DZL), Germany. 7. German Center of Lung Research (DZL), Germany; Department of Pneumology, University of Gießen and Marburg, Gießen and Marburg, Germany. 8. Center for Pulmonary Hypertension and Lung Vascular Diseases, Department of Internal Medicine, Missionsklinik Würzburg, Germany. 9. Centre for Pulmonary Hypertension Hamburg, Pneumology Department, Hamburg-Eppendorf University Hospital, Hamburg, Germany. 10. Internal Medicine II, University Medical Center Regensburg, Regensburg, Germany. 11. Clinic III for Internal Medicine (Cardiology) and Center for Molecular Medicine (CMMC), Cologne, Germany. 12. Klinik für Allgemeine und Interventionelle Kardiologie, Herz- und Diabeteszentrum NRW, Bad Oeynhausen, Germany. 13. Pulmonary Hypertension Unit, Department of Cardiovascular and Respiratory Diseases, Sapienza University of Rome, Rome, Italy. 14. German Center of Lung Research (DZL), Germany; LungenClinic Grosshansdorf, Großhansdorf, Germany. 15. Medical Department I, Carl Gustav Carus University Hospital at the TU Dresden, Dresden, Germany. 16. Department of Pulmonary Medicine, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam Cardiovascular Sciences, Amsterdam, The Netherlands. 17. Department of Internal Medicine II-Cardiology/Pneumology, University of Bonn, Bonn, Germany. 18. Department and Outpatient Department for Internal Medicine, Pneumology/Infectiology, University Medicine Greifswald, Greifswald, Germany. 19. National Heart and Lung Institute, Imperial College London, London, United Kingdom. 20. Department of Pneumology, University Hospital Leuven, Leuven, Belgium. 21. Department of Internal Diseases, Riga Stradiņš University, Riga, Latvia. 22. Department of Cardiology, Royal Free Hospital, London, United Kingdom. 23. Clinic for Pneumology, Zurich University Hospital, Zurich, Switzerland. 24. Department of Cardiology, DRK Kliniken Berlin Westend, Berlin, Germany. 25. Department of Congenital Heart Defects and Pediatric Cardiology, German Heart Centre Munich, Hospital at the Technical University Munich, Munich, Germany. 26. Department of Rheumatology, University Hospital, Zurich, Switzerland.
Abstract
BACKGROUND: Pulmonary hypertension (PH) in COPD is a poorly investigated clinical condition. RESEARCH QUESTION: Which factors determine the outcome of PH in COPD? STUDY DESIGN AND METHODS: We 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). RESULTS: The 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. INTERPRETATION: Patients 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 REGISTRY: ClinicalTrials.gov; No.: NCT01347216; URL: www.clinicaltrials.gov.
BACKGROUND: Pulmonary hypertension (PH) in COPD is a poorly investigated clinical condition. RESEARCH QUESTION: Which factors determine the outcome of PH in COPD? STUDY DESIGN AND METHODS: We 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). RESULTS: The 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. INTERPRETATION: Patients 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 REGISTRY: ClinicalTrials.gov; No.: NCT01347216; URL: www.clinicaltrials.gov.
Authors: Daniel P Cook; Meng Xu; Victoria L Martucci; Jeffrey S Annis; Melinda C Aldrich; Anna R Hemnes; Evan L Brittain Journal: Pulm Circ Date: 2022-01-03 Impact factor: 2.886
Authors: Marius M Hoeper; Christine Pausch; Ekkehard Grünig; Gerd Staehler; Doerte Huscher; David Pittrow; Karen M Olsson; Carmine Dario Vizza; Henning Gall; Oliver Distler; Christian Opitz; J Simon R Gibbs; Marion Delcroix; H Ardeschir Ghofrani; Stephan Rosenkranz; Da-Hee Park; Ralf Ewert; Harald Kaemmerer; Tobias J Lange; Hans-Joachim Kabitz; Dirk Skowasch; Andris Skride; Martin Claussen; Juergen Behr; Katrin Milger; Michael Halank; Heinrike Wilkens; Hans-Jürgen Seyfarth; Matthias Held; Daniel Dumitrescu; Iraklis Tsangaris; Anton Vonk-Noordegraaf; Silvia Ulrich; Hans Klose Journal: Eur Respir J Date: 2022-06-02 Impact factor: 33.795
Authors: Marius M Hoeper; Christine Pausch; Karen M Olsson; Doerte Huscher; David Pittrow; Ekkehard Grünig; Gerd Staehler; Carmine Dario Vizza; Henning Gall; Oliver Distler; Christian Opitz; J Simon R Gibbs; Marion Delcroix; H Ardeschir Ghofrani; Da-Hee Park; Ralf Ewert; Harald Kaemmerer; Hans-Joachim Kabitz; Dirk Skowasch; Juergen Behr; Katrin Milger; Michael Halank; Heinrike Wilkens; Hans-Jürgen Seyfarth; Matthias Held; Daniel Dumitrescu; Iraklis Tsangaris; Anton Vonk-Noordegraaf; Silvia Ulrich; Hans Klose; Martin Claussen; Tobias J Lange; Stephan Rosenkranz Journal: Eur Respir J Date: 2022-07-07 Impact factor: 33.795