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Predicting Survival in Pulmonary Arterial Hypertension Using the REVEAL Database

Raymond Benza


A. J. Foreman

Mardi Gomberg-Maitland


Robert Frantz


D. Miller

C. Gregory Elliott


Michael McGoon


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Conference: 2010 International PHA Conference and Scientific Sessions

Release Date: 06.24.2010

Presentation Type: Abstracts

Benza RL1, Foreman AJ2, Gomberg-Maitland M3, Frantz RP4, Miller DP2, Elliott CG5, McGoon MD4
1. Allegheny General Hospital, Pittsburgh, PA, USA 
2. ICON Clinical Research, Ellicott City, MD, USA 
3. University of Chicago Medical Center, Chicago, IL, USA 
4. Mayo Clinic, Rochester, MN, USA 
5. Intermountain Medical Center, University of Utah, Murray, UT, USA

BACKGROUND: Current clinical estimates of mortality in pulmonary arterial hypertension (PAH) were developed in the 1980s using untreated idiopathic PAH patients in a national registry. We have used the Registry to EValuate Early And Long Term PAH Disease Management (REVEAL) to develop an improved prognostic formula for all PAH subgroups using a combination of factors.

METHODS: All 2716 patients with pulmonary capillary wedge pressure ≤15 mmHg enrolled in the REVEAL Registry from March 30, 2006 through September 11, 2007 were used for this analysis, which fit Cox proportional hazards survival models to the data using stepwise selection with a pre-specified set of candidate variables.

RESULTS: Based on the multivariable model, variables associated with greater than two-fold increase of death were: PVR >32 Wood units (HR 4.1, P<0.001), PAH associated with portal hypertension (HR 3.6, P<0.001), modified New York Heart Association/World Health Organization (NYHA/WHO) functional class (FC) IV (HR 3.1,P<0.001), men >60 years old (HR 2.2, P<0.001), and a family history of PAH (HR 2.2, P=0.012). Other variables associated with a significantly increased risk of death, in decreasing order of magnitude, included BNP >180 pg/mL (HR 1.97, P<0.001), renal insufficiency (HR 1.90, P<0.001), mRAP within the year preceding enrollment >20 mmHg (1.79, P=0.043), 6-MWD <165 m (HR 1.68, P<0.001), resting systolic blood pressure <110 mmHg (HR 1.67, P<0.001), PAH associated with connective tissue disease (HR 1.59, P<0.001), % predicted DLco ≤32% (1.46, P=0.018), modified NYHA/WHO FC III (HR 1.41, P=0.008), resting heart rate >92 bpm (HR 1.39,P=0.005), and the presence of a pericardial effusion (HR 1.35, P=0.014). Four variables conferred increased one-year survival: modified NYHA/WHO FC I (HR 0.42, P=0.039), % predicted DLco ≥80% (HR 0.59, P=0.031), 6-MWD ≥440 m (HR 0.58, P=0.006), and BNP <50 pg/mL (HR 0.50, P=0.003).

CONCLUSIONSA new risk stratification formula has been developed that incorporates PAH subgroups, 6MWD, and updated weighting for hemodynamic variables to more accurately predict survival in patients with PAH in the modern treatment era.