Abby M. Krichman

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.

The changing picture of patients with pulmonary arterial hypertension in the United States: How REVEAL differs from historic and non-US contemporary registries

BACKGROUND REVEAL (The Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management) provides current demographics of patients with group 1 pulmonary arterial hypertension (PAH) in the United States. METHODS A total of 2,967 patients with PAH diagnosed based on right-sided heart catheterization were enrolled in REVEAL between March 2006 and September 2007. Demographics from the REVEAL patient cohort and REVEAL subpopulations (matched by inclusion criteria to other registries) were compared with historic US registry data and other contemporary US and non-US national PAH registries by inclusion criteria, including the National Institutes of Health (NIH) PAH registry and the French PAH registry. RESULTS REVEAL patients matched to NIH registry patients were older at diagnosis (mean ± SE, 44.9 ± 0.6 years vs 36.4 ± 1.1 years; difference, 8.5 ± 1.4; P < .001) and more likely to be women (78.7 ± 1.2% vs 63.1 ± 3.5%; P < .001). REVEAL patients matched to French registry patients had similar age and severity at diagnosis, but REVEAL patients were more likely to be women (79.8 ± 0.8% vs 65.3 ± 1.8%; P < .001) and obese (BMI, ≥ 30 kg/m(2), 32.5 ± 1.0% vs 14.8 ± 1.4%; P < .001), whereas French patients were more likely to have HIV-associated PAH (6.2% vs 2.3%). The female preponderance is similar to that in other US-based contemporary registries. CONCLUSIONS At diagnosis, REVEAL patients were older than NIH registry patients and similar in age to patients enrolled in contemporary registries. Compared with NIH and contemporary European and UK registries, there was a striking preponderance of women, and REVEAL patients were more likely to be obese. These observations and the difference in HIV-associated PAH between REVEAL and other non-US contemporary registries warrant further investigation. TRIAL REGISTRY ClinicalTrials.gov; No.: NCT00370214; URL: clinicaltrials.gov.

Design of the REVEAL Registry for US Patients With Pulmonary Arterial Hypertension

The Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL) has been designed to meet the need for current information about patients with pulmonary arterial hypertension (PAH). The main objectives of REVEAL are to better define and understand PAH and to assess the consequences of treatment strategies. REVEAL is collecting clinically relevant data from 3500 consecutively enrolled patients with confirmed PAH diagnoses. Outcomes will be evaluated longitudinally and compared according to the baseline classification of PAH. The primary outcome for group comparisons will be survival. Collected data include World Health Organization functional class, 6-minute walk distance, cardiopulmonary exercise testing, pulmonary function test results, hemodynamic measurements, functional status, hospitalizations, and death. REVEAL will be the richest source of data on patients with World Health Organization group I PAH.

Analysis of the Lung Allocation Score Estimation of Risk of Death in Patients With Pulmonary Arterial Hypertension Using Data From the REVEAL Registry

Background. Waitlist mortality for patients with pulmonary arterial hypertension (PAH) has not improved after implementation of the lung allocation score (LAS). We analyzed data from patients in the Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL) as a means to compare observed mortality with predicted mortality from the LAS to identify key prognostic parameters that may be incorporated into the LAS to improve waitlist mortality for patients with PAH. Methods. Of the 2967 patients with PAH enrolled in REVEAL, 2327 had all required variables available to compute the LAS. Univariable and multivariable analyses were conducted to compare waitlist survival predicted by the LAS formula with that observed in the REVEAL cohort. Results. Two variables were independently associated with increased mortality compared with that predicted by the LAS in multivariable analysis using a Cox proportional hazards model: mean right atrial pressure (mRAP) more than or equal to 14 mm Hg and 6-min walk test distance (6-MWD) less than or equal to 300 m. A modified LAS system was developed, updating the waitlist survival component of the calculation as follows: modified waitlist exponent equals original waitlist exponent plus 0.7; if mRAP is more than or equal to 14 mm Hg, minus 0.35; for every 100 m of 6-MWD, plus 1.0. Conclusions. The LAS is reevaluated every 6 months after the initial 3-year trial period. Our results suggest that an LAS model that includes both 6-MWD and mRAP better discriminates waitlist urgency for patients with PAH than the current LAS.