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REVEAL Registry: Comparison of Functional Status in Adult Pulmonary Arterial Hypertension (PAH) Patients With and Without Comorbid Medical Conditions

Abby Poms


Michelle Turner

Prieya Wason

Alison Graves

Michael McGoon


Harrison Farber


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Conference: 2009 PH Resource Network Symposium

Release Date: 09.24.2009

Presentation Type: Abstracts

Abby Krichman, BS, RRT1; Michelle Turner, MS2; Prieya Wason, CCRP3; Alison M. Graves Jones, MPH2; Michael D. McGoon, MD4; Harrison W. Farber, MD5
1. Duke University Medical Center, Durham, NC

2. ICON Clinical Research, San Francisco, CA
3. Actelion Pharmaceuticals US, Inc., South San Francisco, CA
4. Mayo Clinic, Rochester, MN
5. Boston University School of Medicine, Boston, MA 

PURPOSE:  To characterize the number and type of comorbid conditions in PAH patients enrolled in the Registry to Evaluate Early And Long-term PAH Disease Management (REVEAL), to determine the effect of the most common comorbid conditions on functional capacity as measured by 6-minute walk test distance (6MWD) and functional class (FC) and to calculate the Charlson Comorbidity Index and examine its association with functional capacity. 

BACKGROUND: REVEAL is a multicenter, observational, U.S. study designed to assess demographic, clinical and management data on patients diagnosed with pulmonary arterial hypertension (PAH). Strengths of the registry are the large number of patients enrolled and the ability to follow patients a minimum of 5 years from enrollment.

METHODS: All consecutive qualified consenting PAH patients are being enrolled at 54 sites in the U.S. Enrollment data include: clinical and treatment history, physical examination, and disease severity assessment.

FINDINGS: 2967 patients were enrolled in the registry between March 2006 and September 2007. For the 2732 patients who were 19 years of age or older at enrollment, the following pre-specified comorbid medical conditions were reported: systemic hypertension, N=1095, 40%; obesity (body mass index ≥30), N=788/2344, 34%; connective tissue disease (CTD), N=765, 28%; clinical depression, N=691, 25%; obstructive airway disease, N=599, 22%; sleep apnea, N=574/2579, 22%; and diabetes, N=340, 12%. In only 7.5% of patients were no comorbidities reported. For all patients, the mean number of comorbidities (excluding PAH) was 4.0 ± 2.8. Among patients exhibiting the pre-specified comorbidities, the mean number of different comorbidities in each patient ranged from an average of 5.2 (in patients with CTD, including CTD as 1 comorbidity and excluding PAH) to an average of 6.1 (in patients with diabetes, including diabetes as 1 comorbidity and excluding PAH). FC at enrollment was significantly worse in patients exhibiting any of the prespecified comorbidities, except for systemic hypertension, compared with patients without the specific comorbidity (all <0.05). Likewise, the mean 6MWD at enrollment was significantly poorer in patients with any of the pre-specified comorbid conditions, including systemic hypertension, than in patients without the specific comorbidity (all <0.05).

IMPLICATIONS: Patients with PAH plus one of the 7 most common comorbidities had significantly worse FC (except systemic hypertension) and 6MWD than patients without the specific comorbid condition.