Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: Results from the COMPERA registry |
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| Authors: | Marius M. Hoeper,Doerte Huscher,H. Ardeschir Ghofrani,Marion Delcroix,Oliver Distler,Christian Schweiger,Ekkehard Grunig,Gerd Staehler,Stephan Rosenkranz,Michael Halank,Matthias Held,Christian Grohé ,Tobias J. Lange,Juergen Behr,Hans Klose,Heinrike Wilkens,Arthur Filusch,Martin Germann,Ralf Ewert,Hans-Juergen Seyfarth,Karen M. Olsson,Christian F. Opitz,Sean P. Gaine,C. Dario Vizza,Anton Vonk-Noordegraaf,Harald Kaemmerer,J. Simon R. Gibbs,David Pittrow |
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| Affiliation: | 1. Department of Respiratory Medicine and German Center of Lung Research, Hannover Medical School, Hannover, Germany;2. Department of Rheumatology and Clinical Immunology, Charité University Hospital, and Epidemiology unit, German Rheumatism Research Centre, Berlin, Germany;3. Department of Pneumology, University of Giessen Lung Center, Giessen, Germany;4. Department of Pneumology, University Hospitals of Leuven, Leuven, Belgium;5. Department of Rheumatology, University Hospital, Zurich, Switzerland;6. Institute for Education in Pharmaceutical Medicine, University Duisburg-Essen, Germany;g University Hospital Heidelberg, Heidelberg, Germany;h Medical Clinic I, Clinic Loewenstein, Loewenstein, Germany;i Clinic III for Internal Medicine (Cardiology), and Center for Molecular Medicine Cologne (CMMC), University of Cologne, Germany;j Internal Medicine I, University Hospital Carl Gustav Carus of Technical University Dresden, Dresden, Germany;k Department of Internal Medicine, Respiratory Medicine and Cardiology, Mission Medical Hospital, Würzburg, Germany;l Department of Respiratory Medicine, ELK Thorax Center, Berlin, Germany;m Department of Internal Medicine II, Division of Pneumology, University Medical Center Regensburg, Regensburg, Germany;n Department of Internal Medicine I, University of Munich and Department of Internal Medicine III, Ruhr-University Bochum, Germany;o University Medical Center Hamburg-Eppendorf, Center of Oncology, Department of Respiratory Medicine, Germany;p Department of Respiratory Medicine, University Hospital Saarland, Homburg, Germany;q Department of Cardiology and Pneumology, University of Heidelberg, Heidelberg, Germany;r Department of Pneumology, University Medical Centre, Freiburg, Germany;s Clinic of Internal Medicine, Department of Respiratory Medicine, Ernst Moritz Arndt University of Greifswald, Germany;t Department of Respiratory Medicine, University of Leipzig, Germany;u Department of Cardiology, DRK Kliniken Berlin Köpenick, Berlin, Germany;v Department of Respiratory Medicine, The Mater Misericordiae University Hospital, Dublin, Ireland;w Department of Cardiovascular and Respiratory Diseases, Sapienza, University of Rome, Rome, Italy;x Department of Pulmonary Diseases, VU University Medical Center, Amsterdam, The Netherlands;y Department of Pediatric Cardiology and Congenital Cardiac Defects, German Heart Centre Munich, Technical University Munich, Germany;z Department of Cardiology, National Heart & Lung Institute, Imperial College London, United Kingdom;aa Institute for Clinical Pharmacology, Medical Faculty, Technical University, Dresden, Germany |
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| Abstract: | BackgroundOriginally reported to occur predominantly in younger women, idiopathic pulmonary arterial hypertension (IPAH) is increasingly diagnosed in elderly patients. We aimed to describe the characteristics of such patients and their survival under clinical practice conditions.MethodsProspective registry in 28 centers in 6 European countries. Demographics, clinical characteristics, hemodynamics, treatment patterns and outcomes of younger (18–65 years) and elderly (> 65 years) patients with newly diagnosed IPAH (incident cases only) were compared.ResultsA total of 587 patients were eligible for analysis. The median (interquartile, [IQR]) age at diagnosis was 71 (16) years. Younger patients (n = 209; median age, 54 [16] years) showed a female-to-male ratio of 2.3:1 whereas the gender ratio in elderly patients (n = 378; median age, 75 [8] years) was almost even (1.2:1). Combinations of PAH drugs were widely used in both populations, albeit less frequently in older patients. Elderly patients were less likely to reach current treatment targets (6 min walking distance > 400 m, functional class I or II). The survival rates 1, 2, and 3 years after the diagnosis of IPAH were lower in elderly patients, even when adjusted for age- and gender-matched survival tables of the general population (p = 0.006 by log-rank analysis).ConclusionsIn countries with an aging population, IPAH is now frequently diagnosed in elderly patients. Compared to younger patients, elderly patients present with a balanced gender ratio and different clinical features, respond less well to medical therapy and have a higher age-adjusted mortality. Further characterization of these patients is required.Clinical trials registration: NCT01347216. |
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| Keywords: | Hypertension Pulmonary Elderly Hemodynamics Survival |
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