Conference: 2008 International PHA Conference and Scientific Sessions
Release Date: 06.20.2008
Presentation Type: Abstracts
Badesch D.B.1, Barst R.J.2, Benza R.L.3, Elliott C.G.4, Farber H.5, Frost A.E.6, Krichman A.7, Liou T.G.8, Raskob G.E.9, Giles S.10, Feldkircher K.10, Miller D.P.11, McGoon,M.D.12
1. University of Colorado and Health Sciences Center, Denver, CO, USA
2. Columbia University Medical Center, New York, NY, USA
3. University of Alabama at Birmingham, Birmingham, AL, USA
4. LDS Hospital, Salt Lake City, UT, USA
5. Boston University School of Medicine, Boston, MA, USA
6. Baylor College of Medicine, Houston, TX, USA
7. Duke University Medical Center, Durham, NC, USA
8. University of Utah, Salt Lake City, UT, USA
9. University of Oklahoma Health Sciences Center, Oklahoma City, OK, USA
10. Actelion Pharmaceuticals US, Inc., USA
11. ICON Clinical Research, USA
12. Mayo Clinic, Rochester, MN, USA
BACKGROUND: 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). At the time of enrollment 8% were receiving no PAH-specific medications, 44% received only oral medications, and 31% received a prostacyclin combined with other medications. PAH-specific medications included: 9%, calcium channel blocker (CCB); 44%, prostacyclin analogue; 47%, endothelin receptor antagonist; and 46%, phosphodiesterase inhibitor.
CONCLUSIONS: 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.