PURPOSE: The Registry to EValuate Early And Long-term PAH Disease Management (REVEAL) is a multicenter, observational, U.S.-based study that is designed to meet the need for current and reliable information about the demographics, clinical course and management of patients diagnosed with pulmonary arterial hypertension (PAH).
METHODS: Consecutive patients are screened, and all consenting patients with PAH diagnosed according to specific hemodynamic criteria are being enrolled at 50 sites in the U.S. Patients will be followed for a minimum of five years. Demographic data are being gathered at the time of enrollment.
RESULTS: 1226 patients are currently enrolled in the registry; 8% are newly diagnosed with PAH and 92% have previously diagnosed PAH. Most of the patients are female (78%), and the median age is 53 years. Approximately half of the patients have idiopathic PAH (IPAH, 46%), and half have PAH associated with various conditions (APAH [50%]). 49% of patients with APAH have collagen vascular disease and 22% have congenital heart disease. Most of the patients are in NYHA/WHO functional classes [FC] II or III (FC I, 8%; FC II, 38%; FC III, 49%; FC IV, 6%). Thirty percent of patients are currently employed or in school; most are living independently (95%). The mean (±SD) 6-minute walk distance (6MWD) by FC is 453 ± 119 m (FC I), 426 ± 101 m (FC II), 326 ± 114 m (FC III), and 262 ± 126 m (FC IV).
CONCLUSION: The baseline demographics of patients enrolled in the REVEAL registry suggest that the patients with PAH seen currently in clinical practice are older, predominantly female, and equally likely to have IPAH or APAH. The data set illustrates the relationship between FC and 6MWD.
CLINICAL IMPLICATIONS: The REVEAL Registry will provide a large data set on PAH, including patient demographics, clinical course, and current therapeutic management. These data will complement the information obtained from prospective clinical trials, permitting the investigative community to address an array of clinically important questions.
DISCLOSURE: David Badesch, No Product/Research Disclosure Information; Grant monies (from sources other than industry) David Badesch has received grant/research support from the National Institutes of Health; Grant monies (from industry related sources) David Badesch has received grant/research support from GlaxoSmithKline, United Therapeutics/Lung Rx, Actelion, Lilly/ICOS, Encysive, Pfizer, Myogen/Gilead and CoTherix; Employee Scott Giles is an employee of Actelion; Fiduciary position (of any organization, association, society, etc, other than ACCP David Badesch sits on the Pulmonary Hypertension Association Board of Directors and the American Thoracic Society Board of Directors.; Consultant fee, speaker bureau, advisory committee, etc. David Badesch has served as a consultant for GlaxoSmithKline, Actelion, Myogen/Gilead, Encysive, CoTherix, Pfizer, United Therapeutics, Mondo-Biotech, Biogen IDEC, PR Pharmaceuticals, Forrest Labs, Scios, Amgen, Biovale Pharmaceuticals/Clarus Health and Johnson & Johnson; has served on a speaker bureau for GlaxoSmithKline, Actelion, Encysive, Myogen, CoTherix, United Therapeutics and Pfizer; has served on an advisory committee for GlaxoSmithKline, Actelion, Myogen/Gilead, Encysive, CoTherix, Pfizer, United Therapeutics, Mondo-Biotech and Biogen IDEC. Raymond Benza has received honoraria from Actelion, Lung Rx and United Therapeutics. Abby Krichman is a consultant for Actelion, Lung Rx, United Therapeutics and MedaCorp. Gary Raskob has served as a Steering Committee member and consultant for Actelion.