@article{ba075dcb29ea4489b557aadf4aa76b7b,
title = "The changing picture of patients with pulmonary arterial hypertension in the United States: How REVEAL differs from historic and non-US contemporary registries",
abstract = "Background: REVEAL (The Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management) provides current demographics of patients with group 1 pulmonary arterial hypertension (PAH) in the United States. Methods: A total of 2,967 patients with PAH diagnosed based on right-sided heart catheterization were enrolled in REVEAL between March 2006 and September 2007. Demographics from the REVEAL patient cohort and REVEAL subpopulations (matched by inclusion criteria to other registries) were compared with historic US registry data and other contemporary US and non-US national PAH registries by inclusion criteria, including the National Institutes of Health (NIH) PAH registry and the French PAH registry. Results: REVEAL patients matched to NIH registry patients were older at diagnosis (mean ± SE, 44.9 ± 0.6 years vs 36.4 ± 1.1 years; difference, 8.5 ± 1.4; P<.001) and more likely to be women (78.7 ± 1.2% vs 63.1 ± 3.5%; P<.001). REVEAL patients matched to French registry patients had similar age and severity at diagnosis, but REVEAL patients were more likely to be women (79.8 ± 0.8% vs 65.3 ± 1.8%; P<.001) and obese (BMI, ≥ 30 kg/m2, 32.5 ± 1.0% vs 14.8 ± 1.4%; P<.001), whereas French patients were more likely to have HIV-associated PAH (6.2% vs 2.3%). The female preponderance is similar to that in other US-based contemporary registries. Conclusions: At diagnosis, REVEAL patients were older than NIH registry patients and similar in age to patients enrolled in contemporary registries. Compared with NIH and contemporary European and UK registries, there was a striking preponderance of women, and REVEAL patients were more likely to be obese. These observations and the difference in HIV-associated PAH between REVEAL and other non-US contemporary registries warrant further investigation. Trial registry: ClinicalTrials.gov; No.: NCT00370214; URL: clinicaltrials.gov.",
author = "Frost, {Adaani E.} and Badesch, {David B.} and Barst, {Robyn J.} and Benza, {Raymond L.} and {Gregory Elliott}, C. and Farber, {Harrison W.} and Abby Krichman and Liou, {Theodore G.} and Raskob, {Gary E.} and Prieya Wason and Kathleen Feldkircher and Michelle Turner and McGoon, {Michael D.}",
note = "Funding Information: Funding/Support: This study was funded by Actelion Pharmaceuticals US, Inc. Funding Information: The first registry for PAH, known as the Patient Registry for the Characterization of Primary Pulmonary Hypertension, 5 began in 1981 and was sponsored by the National Institutes of Health (NIH) National Heart, Lung, and Blood Institute (NHLBI). It included 187 patients and provided the foundation for subsequent studies of both PAH and other pulmonary vascular diseases. More recent US registries include the large, single-center Pulmonary Hypertension Connection (PHC) registry (1982-2006) (N = 578) 6 and the Surveillance of Pulmonary Hypertension in America registry (1998-2001) (1,180 incident enrollees with pulmonary hypertension, 7 including 346 patients with group 1 PAH evaluated in the context of anorexigen drug exposure). National registries in Scotland and France have provided more current information on the nature of PAH in those countries. The Scottish registry used data from the Scottish Morbidity Record (1986-2001) (374 incidence cases) and the national specialist center for PAH management known as the Scottish Pulmonary Vascular Unit (1997-2005), whereas the French Network on PAH studied current epidemiologic trends (2002-2003) (N = 674). 8,9 Despite useful contributions to the PAH literature, the Surveillance of North American Pulmonary Hypertension (N = 205), 10 a Swiss registry, 11,12 and a Chinese registry 13 were excluded from comparison in the current study because of lack of diagnostic RHC and inclusion of patients with conditions other than group 1 PAH. Funding Information: Financial/nonfinancial disclosures: The authors have reported to CHEST the following conflicts of interest: Dr Frost serves as a consultant for Gilead and Actelion; has received honoraria from Gilead, Actelion, and Pfizer; has provided expert testimony on diet pill litigation; has received grants from Gilead and Actelion and grants to Baylor for Institutional Review Board-approved research; and has received honoraria for her service on the REVEAL Steering Committee, which is supported by Actelion. Dr Badesch has received honoraria for service on steering committees and advisory boards for Actelion/CoTherix, Gilead/Myogen, Encysive Pharmaceuticals, Ikaria, Arena, Bayer, Pfizer, GlaxoSmithKline, Lung Rx, United Therapeutics, Eli Lilly & Co/ICOS, Biogen Idec, and MondoBIOTECH; has received grants from Actelion/CoTherix, Gilead/Myogen, Encysive Pharmaceuticals, Bayer, Novartis, Pfizer, United Therapeutics, Lung Rx, Eli Lilly & Co/ICOS, and NIH/NHLBI; and has received honoraria for his service on the REVEAL Steering Committee, which is supported by Actelion. Dr Barst serves as a consultant for and has received honoraria from Actelion, Bayer, GlaxoSmithKline, GeneraMedix, Gilead, Eli Lilly & Co, MondoBIOTECH, NIH/NHLBI, Novartis, and Pfizer; has provided expert testimony on diet pill litigation for the plaintiffs; has received grants from Actelion, Gilead, Eli Lilly & Co, NIH/NHLBI, Novartis, Pfizer, and United Therapeutics; and has received honoraria for her service on the REVEAL Steering Committee, which is supported by Actelion. Dr Benza has received honoraria from Actelion, United Therapeutics, and Gilead; has received or is pending receipt of grants from Actelion, United Therapeutics, Gilead, and Lung Rx; and has received honoraria for his service on the REVEAL Steering Committee, which is supported by Actelion. Dr Elliott is employed by Intermountain Healthcare, which has received grant support (Dr Elliott as principal investigator) during the past 5 years from Actelion, Pfizer, Encysive Pharmaceuticals, and United Therapeutics, and has received honoraria for his service on the REVEAL Steering Committee, which is supported by Actelion. Dr Farber serves as a consultant and is on the speaker's bureau for Actelion and has received honoraria for his service on the REVEAL Steering Committee, which is supported by Actelion. Ms Krichman has received honoraria for her service on the REVEAL Steering Committee, which is supported by Actelion. Dr Liou has received grants from NIH/NHLBI, the Margolis Family Foundation of Utah, and the Cystic Fibrosis Foundation; has been the site principal investigator for studies of cystic fibrosis and its treatment for the Therapeutic Development Network of the Cystic Fibrosis Foundation, Altus, Axcan Scandipharm, Bayer, Boehringer-Ingelheim, Genentech, Gilead, Inspire, Kalobios, MPEX, Novartis, and Vertex; and has received honoraria for his service on the REVEAL Steering Committee, which is supported by Actelion. Dr Raskob serves as a consultant and receives honoraria from Actelion, Bayer, Bristol-Myers Squibb, Daiichi Sankyo, Inc, GlaxoSmithKline, Johnson & Johnson, Pfizer, sanofi-aventis, Takeda, TOPP, and Thrombogenics and has received honoraria for his service on the REVEAL Steering Committee, which is supported by Actelion. Ms Wason is employed by Actelion Pharmaceuticals US, Inc. Dr Feldkircher is employed by Actelion Pharmaceuticals US, Inc. Ms Turner is employed by ICON Clinical Research, a company that receives research support from Actelion and other pharmaceutical companies. Dr McGoon serves as a consultant with Actelion, Gilead, Lung Rx, and Medtronic; has received grants from Gilead and Medtronic; and has received honoraria for his service on the REVEAL Steering Committee, which is supported by Actelion. ",
year = "2011",
month = jan,
day = "1",
doi = "10.1378/chest.10-0075",
language = "English (US)",
volume = "139",
pages = "128--137",
journal = "CHEST",
issn = "0012-3692",
publisher = "American College of Chest Physicians",
number = "1",
}