Pulmonary Arterial Hypertension Baseline Characteristics From the REVEAL Registry

被引:894
作者
Badesch, David B. [1 ,2 ]
Raskob, Gary E. [3 ]
Elliott, C. Greg [4 ,5 ]
Krichman, Abby M. [6 ]
Farber, Harrison W. [7 ]
Frost, Adaani E. [8 ]
Barst, Robyn. J. [9 ]
Benza, Raymond L. [10 ]
Liou, Theodore G. [11 ]
Turner, Michelle [12 ]
Giles, Scott [13 ]
Feldkircher, Kathy [13 ]
Miller, Dave P. [12 ]
McGoon, Michael D. [14 ]
机构
[1] Univ Colorado, Div Pulm Sci & Crit Care Med, Denver, CO 80202 USA
[2] Univ Colorado, Div Cardiol, Denver, CO 80202 USA
[3] Univ Oklahoma, Hlth Sci Ctr, Coll Publ Hlth, Oklahoma City, OK USA
[4] Intermt Med Ctr, Dept Med, Salt Lake City, UT USA
[5] Univ Utah, Salt Lake City, UT USA
[6] Duke Univ, Med Ctr, Pulm Vasc Dis Ctr, Durham, NC USA
[7] Boston Univ, Med Ctr, Pulm Hypertens Ctr, Boston, MA USA
[8] Baylor Coll Med, Dept Med, Houston, TX 77030 USA
[9] Columbia Univ, Coll Phys & Surg, New York, NY USA
[10] Allegheny Gen Hosp, Gerald McGinnis Cardiovasc Inst, Pittsburgh, PA 15212 USA
[11] Univ Utah, Dept Pulm, Salt Lake City, UT USA
[12] ICON Clin Res, San Francisco, CA USA
[13] Actel Pharmaceut US Inc, San Francisco, CA USA
[14] Mayo Clin, Div Cardiovasc Dis, Rochester, MN USA
关键词
CONTINUOUS INTRAVENOUS EPOPROSTENOL; INHALED ILOPROST; CHILDHOOD-ONSET; THYROID-DISEASE; HIGH PREVALENCE; UNITED-STATES; THERAPY; BOSENTAN; HYPOTHYROIDISM; PROSTACYCLIN;
D O I
10.1378/chest.09-1140
中图分类号
R4 [临床医学];
学科分类号
1002 ; 100602 ;
摘要
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 PAR 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) <= 18 mm Hg, and pulmonary vascular resistance >= 240 dynes.s.cm(-5). Patients meeting the traditional hemodynamic definition (PCWP <= 15 mm Hg) were compared with those with a PCWP of 16 to IS 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 IS 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. CHEST 2010; 137(2):376-387
引用
收藏
页码:376 / 387
页数:12
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