title: Pulmonary arterial hypertension: epidemiology and registries.
abstract: 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.
authors.affiliation: Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota. Electronic address: mmcgoon@mayo.edu.
authors.forename: Michael D
authors.initials: MD
authors.lastname: McGoon
authors.affiliation: Division of Cardiovascular Diseases, Allegheny General Hospital, Pittsburgh, Pennsylvania.
authors.forename: Raymond L
authors.initials: RL
authors.lastname: Benza
authors.affiliation: Cardiology Department and Spanish Cardiovascular Research Network, Hospital Universitario, Madrid, Spain.
authors.forename: Pilar
authors.initials: P
authors.lastname: Escribano-Subias
authors.affiliation: Thrombosis Medicine Center, State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, Peking Union Medical College and Chinese Academy Medical Science, Beijing, China.
authors.forename: Xin
authors.initials: X
authors.lastname: Jiang
authors.affiliation: ICON Clinical Research, Medical Affairs Statistical Analysis, San Francisco, California.
authors.forename: Dave P
authors.initials: DP
authors.lastname: Miller
authors.affiliation: Scottish Pulmonary Vascular Unit, Regional Heart and Lung Center, Glasgow, United Kingdom.
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Peacock
authors.affiliation: Pulmonary Vascular Diseases Unit, Papworth Hospital, Cambridge, United Kingdom.
authors.forename: Joanna
authors.initials: J
authors.lastname: Pepke-Zaba
authors.affiliation: Cardiopulmonary Department, National Heart Institute, Mexico City, Mexico.
authors.forename: Tomas
authors.initials: T
authors.lastname: Pulido
authors.affiliation: Section of Cardiology, University of Chicago, Chicago, Illinois.
authors.forename: Stuart
authors.initials: S
authors.lastname: Rich
authors.affiliation: Clinic III for Internal Medicine, Department of Cardiology, Heart Center at the University of Cologne, Cologne, Germany.
authors.forename: Stephan
authors.initials: S
authors.lastname: Rosenkranz
authors.affiliation: Centre for Clinical Epidemiology, Jewish General Hospital, Department of Epidemiology and Biostatistics, McGill University, Montreal, Quebec, Canada.
authors.forename: Samy
authors.initials: S
authors.lastname: Suissa
authors.affiliation: Universite Paris-Sud, Inserm U999, LabEx LERMIT, AP-HP, DHU Thorax Innovation, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin Bicêtre, France.
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
mesh_terms: D000818:Animals; D065627:Familial Primary Pulmonary Hypertension; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000453:epidemiology*; D011237:Predictive Value of Tests; D012042:Registries / Q000592:standards
keywords: 6-min walk distance; 6MWD; CRF; CTEPH; NIH; National Institutes of Health; PAH; PH; PPH; case report form; chronic thromboembolic pulmonary hypertension; databases; epidemiology; primary pulmonary hypertension; pulmonary arterial hypertension; pulmonary hypertension; registries