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 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

860 Citations (Scopus)

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

Fingerprint

Disease Management
Pulmonary Hypertension
Registries
Confidence Intervals
Survival
Blood Pressure
Connective Tissue Diseases
Atrial Pressure
Pericardial Effusion
Mortality
Brain Natriuretic Peptide
Portal Hypertension
Carbon Monoxide
Vascular Resistance
Renal Insufficiency
Heart Rate
Research

Keywords

  • Prognosis
  • Pulmonary arterial hypertension
  • Risk factors
  • Survival

ASJC Scopus subject areas

  • Physiology (medical)
  • Cardiology and Cardiovascular Medicine

Cite this

Predicting survival in pulmonary arterial hypertension : Insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management (REVEAL). / Benza, Raymond L.; Miller, Dave P.; Gomberg-Maitland, Mardi; Frantz, Robert; Foreman, Aimee J.; Coffey, Christopher S.; Frost, Adaani; Barst, Robyn J.; Badesch, David B.; Elliott, C. Gregory; Liou, Theodore G.; McGoon, Michael D.

In: Circulation, Vol. 122, No. 2, 13.07.2010, p. 164-172.

Research output: Contribution to journalArticle

Benza, RL, Miller, DP, Gomberg-Maitland, M, Frantz, R, Foreman, AJ, Coffey, CS, Frost, A, Barst, RJ, Badesch, DB, Elliott, CG, Liou, TG & McGoon, MD 2010, 'Predicting survival in pulmonary arterial hypertension: Insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management (REVEAL)', Circulation, vol. 122, no. 2, pp. 164-172. https://doi.org/10.1161/CIRCULATIONAHA.109.898122
Benza, Raymond L. ; Miller, Dave P. ; Gomberg-Maitland, Mardi ; Frantz, Robert ; Foreman, Aimee J. ; Coffey, Christopher S. ; Frost, Adaani ; Barst, Robyn J. ; Badesch, David B. ; Elliott, C. Gregory ; Liou, Theodore G. ; McGoon, Michael D. / Predicting survival in pulmonary arterial hypertension : Insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management (REVEAL). In: Circulation. 2010 ; Vol. 122, No. 2. pp. 164-172.
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