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REVEAL Registry: Baseline Characteristics of Patients with Childhood-Onset PAH

Robyn Barst


David Badesch


Raymond Benza


C. Gregory Elliott


Harrison Farber


Adaani Frost

Abby Poms


T. Liou

G. Raskob

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Conference: 2008 International PHA Conference and Scientific Sessions

Release Date: 06.20.2008

Presentation Type: Abstracts

Barst R.J.1, Ivy D.2, Badesch D.B.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. Columbia University Medical Center, New York, NY, USA
2. University of Colorado and Health Sciences Center, Denver, CO, 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

BACKGROUNDThe 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).

METHODSConsecutive 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.

RESULTS184 patients with childhood-onset PAH (age at diagnosis <18 years) are currently enrolled in the registry; 5% are newly diagnosed with PAH and 95% have previously diagnosed PAH.  Most of the patients are female (65%), the mean age at diagnosis is 7.1 ± 5.4 years, and the mean age at enrollment is 14.3 ± 8.5 years. Approximately half of the patients have idiopathic PAH (IPAH, 49%), and half have PAH associated with various conditions (APAH [43%]). 88% of patients with APAH have congenital heart disease.  Most of the patients are in NYHA/WHO functional class [FC] II (FC I, 22%; FC II, 52%; FC III, 24%; FC IV, 2%).  The mean (±SD) 6-minute walk distance (6MWD) at enrollment is 432 ± 134 m.  The primary physician at first presentation of initial symptoms is usually a cardiologist (65%).

CONCLUSIONS: The baseline demographics of patients enrolled in the REVEAL registry suggest that the patients with childhood-onset PAH are equally likely to have IPAH or APAH. The patients with APAH primarily have PAH associated with congenital heart disease.  Approximately three-quarters of the patients with childhood-onset PAH are in NYHA FC I or II.