Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry.
BACKGROUND
The Registry to EValuate Early And Long-term pulmonary arterial hypertension disease management (REVEAL Registry) was established to provide updated characteristics of patients with pulmonary arterial hypertension (PAH) and to improve diagnosis, treatment, and management.


METHODS
Fifty-four US centers enrolled consecutively screened patients with World Health Organization group I PAH who met expanded hemodynamic criteria of mean pulmonary arterial pressure (PAP) > 25 mm Hg at rest (30 mm Hg with exercise), pulmonary capillary wedge pressure (PCWP) <or= 18 mm Hg, and pulmonary vascular resistance >or= 240 dynes x s x cm(-5). Patients meeting the traditional hemodynamic definition (PCWP <or= 15 mm Hg) were compared with those with a PCWP of 16 to 18 mm Hg.


RESULTS
Between March 2006 and September 2007, 2,967 patients enrolled. Among 2,525 adults meeting traditional hemodynamic criteria, the mean age was 53 +/- 14 years, and 2,007 (79.5%) were women. The mean duration between symptom onset and diagnostic catheterization was 2.8 years, and 1,008 (41.3%) patients were treated with more than one pulmonary vascular-targeted medication. Compared with patients meeting the traditional hemodynamic definition of PAH, patients with a PCWP of 16 to 18 mm Hg were older, more obese, had a lower 6-min walk distance, and had a higher incidence of systemic hypertension, sleep apnea, renal insufficiency, and diabetes.


CONCLUSIONS
Patients in the REVEAL Registry are older and more often female than in previous descriptions. Delays between symptom onset and diagnostic catheterization persist. Many treatment regimens are fundamentally empirical, and data will be required to determine outcomes, improve risk stratification, and develop and validate more precise prognostic tools. Patients with PCWP of 16 to 18 mm Hg differ in a number of important respects from those meeting the traditional hemodynamic definition of PAH.
Badesch
David B
DB
Divisions of Pulmonary Sciences and Critical Care Medicine, and Cardiology, University of Colorado Denver, Denver, CO, USA. david.badesch@ucdenver.edu
Raskob
Gary E
GE
Elliott
C Greg
CG
Krichman
Abby M
AM
Farber
Harrison W
HW
Frost
Adaani E
AE
Barst
Robyn J
RJ
Benza
Raymond L
RL
Liou
Theodore G
TG
Turner
Michelle
M
Giles
Scott
S
Feldkircher
Kathy
K
Miller
Dave P
DP
McGoon
Michael D
MD
D000293:Adolescent; D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D006328:Cardiac Catheterization; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000453:epidemiology* / Q000503:physiopathology; D015994:Incidence; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D011669:Pulmonary Wedge Pressure / Q000502:physiology*; D012042:Registries; D014655:Vascular Resistance / Q000502:physiology*; D055815:Young Adult
D000959:Antihypertensive Agents