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

Research output: Contribution to journalArticle

933 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)164-172
Number of pages9
JournalCirculation
Volume122
Issue number2
DOIs
StatePublished - Jul 13 2010

Keywords

  • Prognosis
  • Pulmonary arterial hypertension
  • Risk factors
  • Survival

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

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    Benza, R. L., Miller, D. P., Gomberg-Maitland, M., Frantz, R. P., Foreman, A. J., Coffey, C. S., Frost, A., Barst, R. J., Badesch, D. B., Elliott, C. G., Liou, T. G., & McGoon, M. D. (2010). Predicting survival in pulmonary arterial hypertension: Insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management (REVEAL). Circulation, 122(2), 164-172. https://doi.org/10.1161/CIRCULATIONAHA.109.898122