Predicting Survival in Pulmonary Arterial Hypertension: Insights From the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL)

Raymond L Benza; Dave P Miller; Mardi Gomberg-Maitland; Robert P Frantz; Aimee J Foreman; Christopher S Coffey; Adaani Frost; Robyn J Barst; David B Badesch; C. Gregory Elliott; Theodore G Liou; Michael D McGoon

doi:10.1161/CIRCULATIONAHA.109.898122

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Predicting Survival in Pulmonary Arterial Hypertension: Insights From the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL)

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Predicting Survival in Pulmonary Arterial Hypertension: Insights From the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL)

Raymond L Benza, Dave P Miller, Mardi Gomberg-Maitland, Robert P Frantz, Aimee J Foreman, Christopher S Coffey, Adaani Frost, Robyn J Barst, David B Badesch, C. Gregory Elliott, …

Circulation (New York, N.Y.), Vol.122(2), pp.164-172

07/13/2010

DOI: 10.1161/CIRCULATIONAHA.109.898122

PMID: 20585012

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Abstract

Background-: Factors that determine survival in pulmonary arterial hypertension (PAH) drive clinical management. A quantitative survival prediction tool has not been established for research or clinical use. Methods and results-: Data from 2716 patients with PAH enrolled consecutively in the US Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) were analyzed to assess predictors of 1-year survival. We identified independent prognosticators of survival and derived a multivariable, weighted risk formula for clinical use. One-year survival from the date of enrollment was 91.0% (95% confidence interval [CI], 89.9 to 92.1). In a multivariable analysis with Cox proportional hazards, variables independently associated with increased mortality included pulmonary vascular resistance >32 Wood units (hazard ratio [HR], 4.1; 95% CI, 2.0 to 8.3), PAH associated with portal hypertension (HR, 3.6; 95% CI, 2.4 to 5.4), modified New York Heart Association/World Health Organization functional class IV (HR, 3.1; 95% CI, 2.2 to 4.4), men >60 years of age (HR, 2.2; 95% CI, 1.6 to 3.0), and family history of PAH (HR, 2.2; 95% CI, 1.2 to 4.0). Renal insufficiency, PAH associated with connective tissue disease, functional class III, mean right atrial pressure, resting systolic blood pressure and heart rate, 6-minute walk distance, brain natriuretic peptide, percent predicted carbon monoxide diffusing capacity, and pericardial effusion on echocardiogram all predicted mortality. Based on these multivariable analyses, a prognostic equation was derived and validated by bootstrapping technique. Conclusions-: We identified key predictors of survival based on the patient's most recent evaluation and formulated a contemporary prognostic equation. Use of this tool may allow the individualization and optimization of therapeutic strategies. Serial follow-up and reassessment are warranted. © 2010 American Heart Association, Inc.

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Title: Subtitle: Predicting Survival in Pulmonary Arterial Hypertension: Insights From the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL)
Creators: Raymond L Benza - From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G
Dave P Miller - From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G
Mardi Gomberg-Maitland - From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G
Robert P Frantz - From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G
Aimee J Foreman - From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G
Christopher S Coffey - From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G
Adaani Frost - From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G
Robyn J Barst - From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G
David B Badesch - From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G
C. Gregory Elliott - From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G
Theodore G Liou - From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G
Michael D McGoon - From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G
Resource Type: Journal article
Publication Details: Circulation (New York, N.Y.), Vol.122(2), pp.164-172
DOI: 10.1161/CIRCULATIONAHA.109.898122
PMID: 20585012
NLM abbreviation: Circulation
ISSN: 0009-7322
eISSN: 1524-4539
Publisher: Lippincott Williams & Wilkins
Language: English
Date published: 07/13/2010
Academic Unit: Biostatistics
Record Identifier: 9984214819902771

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