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.
| Original language | English (US) |
|---|---|
| Pages (from-to) | D51-D59 |
| Journal | Journal of the American College of Cardiology |
| Volume | 62 |
| Issue number | 25 SUPPL. |
| DOIs | |
| State | Published - Dec 24 2013 |
Funding
Dr. McGoon has received institutional grants for studies in which he was the primary investigator from and Medtronic and Gilead; participated in speaking activities for Actelion, Gilead (funded conferences, not speakers' bureaus); was a consultant for Actelion; was the chair of the REVEAL Registry and on the data adjudication committees; on the Data Safety Monitoring Board of Gilead and GlaxoSmithKline; and is on the Advisory Committee of Lung LLC. Dr. Benza has contracted research for Acetlion, Bayer, Gilead, GeNO, Ikaria, and United Therapeutics; and is a consultant for Bayer and United Therapeutics. Dr. Escribano-Subias reports that the Spanish registry of PH is sponsored by a Bayer Schering Pharma educational grant; has received honoraria for sitting on advisory boards and taking at sponsored symposia from Actelion, GlaxoSmithKline, United Therapeutics, Pfizer, Bayer and Ferrer; and has received institutional grants for performing RCTs by the same companies. D. P. Miller is an employee of Icon Clinical Research, which receives research funding from pharmaceutical and biotechnology companies . Dr. Peacock has received honoraria for speaking at meetings (nonpromotional) from Actelion, Bayer, Eli Lilly, GlaxoSmithKline, Novartis, Pfizer, and United Therapeutics; travel assistance to conferences from Actelion, Bayer, Eli Lilly, GlaxoSmithKline, Novartis, Pfizer, and United Therapeutics; research grants (educational only) from Actelion and Bayer ; and has served on the advisory boards of Actelion, Bayer, Eli Lilly, GlaxoSmithKline, Novartis, and Pfizer. Dr. Pepke-Zaba has received reimbursement of travel expenses to congresses and speakers’ fees from Actelion, Pfizer, GlaxoSmithKline, Bayer; has served on the advisory boards of Actelion, Bayer, and GlaxoSmithKline; and has received funds for research and education from Actelion, Pfizer, GlaxoSmithKline, and Bayer . Dr. Pulido has received honoraria for serving as a consultant for Actelion, Bayer, and Pfizer; has received research grants (institutional) from Actelion, Bayer, Gilead, Lilly, Pfizer, and United Therapeutics ; has received honoraria for serving on the advisory boards of Actelion and Bayer; and has received lecture fees from Actelion, Bayer, and Pfizer. Dr. Rosenkrantz has received speaker fees and/or renumerations for consulting from Acetelion, Bayer, GlaxoSmithKline, Lilly, Novartis, Pfizer, and United Therapeutics; and research grants from Actelion, Bayer, Novartis, Pfizer, and United Therapeutics . Dr. Suissa has participated in advisory meetings or as a conference speaker for Actelion, AstraZeneca, Bayer, Boehringer- Ingelheim, GlaxoSmithKline, Merck, Novartis, and Pfizer. Dr. Humbert has been a consultant for and a member of the advisory board of Actelion, Aires, Bayer, GlaxoSmithKline, Novartis, Pfizer, and United Therapeutics as well as being an investigator in trials involving these companies. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Keywords
- databases
- epidemiology
- pulmonary hypertension
- registries
ASJC Scopus subject areas
- Cardiology and Cardiovascular Medicine
