ISSN 1016-5169 | E-ISSN 1308-4488
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Pulmonary arterial hypertension: epidemiology and registries [Turk Kardiyol Dern Ars]
Turk Kardiyol Dern Ars. 2014; 42(1): 67-77

Pulmonary arterial hypertension: epidemiology and registries

Michael D. Mcgoon1, Raymond L. Benza2, Pilar Escribano-subias3, Xin Jiang4, Dave P. Miller5, Andrew J. Peacock6, Joanna Pepke-zaba7, Tomas Pulido8, Stuart Rich9, Stephan Rosenkranz10, Samy Suissa11, Marc Humbert12
1Mayo Clinic Department of Cardiovascular Diseases, Rochester, Minnesota, USA
2Allegheny General Hospital, Department of Cardiovascular Diseases, Pittsburgh, USA
3Madrid University Hospital, Department of Cardiology and the Spanish Cardiovascular Research Network, Spain
4Thrombosis Medical Center, State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, Peking Union University School of Medicine and Medical Sciences Academy of Sciences of China, Beijing, China
5ICON Clinical Research, Medical Affairs Statistical Analysis, San Francisco, California
6Scottish Pulmonary Vascular Unit, Regional Heart and Lung Centre, Glasgow, England
7Papworth Hospital, Pulmonary Vascular Disease Unit, Cambridge, England
8National Heart Institute Cardiopulmonary Department, Mexico City, Mexico
9University of Chicago, Department of Cardiology, Chicago, Illinois
10Cologne University Clinic of Internal Medicine III, Department of Cardiology, Heart Center, Germany
11Center for Clinical Epidemiology, Jewish General Hospital, Department of Epidemiology and Biostatistics, McGill University, Montreal, Quebec, Canada
12Paris-Sud University, Inserm U999, LABEX Lermit, AP-HP, Du Thorax Innovation, Pneumology Service Bicêtre Hospital, Le Kremlin Bicetre, France

Registries of patients with pulmonary arterial hypertension (PAH) have been instrumental in characterizing the presentation and natural history of the disease and provide a basis for prognostication. Since the initial accumulation of data conducted in the 1980s, subsequent registry databases have yielded information about the demographic factors, treatment, and survival of patients and have permitted comparisons between populations in different eras and environments. Inclusion of patients with all subtypes of PAH has also allowed comparisons of these subpopulations. We describe herein the basic methodology by which PAH registries have been conducted, review key insights provided by registries, summarize issues related to interpretation and comparison of the results, and discuss the utility of data to predict survival outcomes. Potential sources of bias, particularly related to the inclusion of incident and/or prevalent patients and missing data, are addressed. A fundamental observation of current registries is that survival in the modern treatment era has improved compared with that observed previously and that outcomes among PAH subpopulations vary substantially. Continuing systematic clinical surveillance of PAH will be important as treatment evolves and as understanding of mechanisms advance. Considerations for future directions of registry studies include enrollment of a broader population of patients with pulmonary hypertension of all clinical types and severity and continued globalization and collaboration of registry databases. (J Am Coll Cardiol 2013;62: D51–9) ©2013 by the American College of Cardiology Foundation.

Keywords: Databases, epidemiology, pulmonary hypertension, registries

How to cite this article
Michael D. Mcgoon, Raymond L. Benza, Pilar Escribano-subias, Xin Jiang, Dave P. Miller, Andrew J. Peacock, Joanna Pepke-zaba, Tomas Pulido, Stuart Rich, Stephan Rosenkranz, Samy Suissa, Marc Humbert. Pulmonary arterial hypertension: epidemiology and registries. Turk Kardiyol Dern Ars. 2014; 42(1): 67-77

Corresponding Author: Michael D. Mcgoon, United States
Manuscript Language: Turkish


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