title: Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL).
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.
  
  
  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. Clinical Trial Registration- URL: http://www.clinicaltrials.gov. Unique identifier: NCT00370214.
authors.affiliation: The Gerald McGinnis Cardiovascular Institute, Allegheny General Hospital, Pittsburgh, PA 15212, USA. rbenza@wpahs.org
authors.forename: Raymond L
authors.initials: RL
authors.lastname: Benza
authors.forename: Dave P
authors.initials: DP
authors.lastname: Miller
authors.forename: Mardi
authors.initials: M
authors.lastname: Gomberg-Maitland
authors.forename: Robert P
authors.initials: RP
authors.lastname: Frantz
authors.forename: Aimee J
authors.initials: AJ
authors.lastname: Foreman
authors.forename: Christopher S
authors.initials: CS
authors.lastname: Coffey
authors.forename: Adaani
authors.initials: A
authors.lastname: Frost
authors.forename: Robyn J
authors.initials: RJ
authors.lastname: Barst
authors.forename: David B
authors.initials: DB
authors.lastname: Badesch
authors.forename: C Gregory
authors.initials: CG
authors.lastname: Elliott
authors.forename: Theodore G
authors.initials: TG
authors.lastname: Liou
authors.forename: Michael D
authors.initials: MD
authors.lastname: McGoon
mesh_terms: D000328:Adult; D000368:Aged; D001794:Blood Pressure; D002248:Carbon Monoxide / Q000097:blood; D018572:Disease-Free Survival; D005260:Female; D006339:Heart Rate; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood / Q000401:mortality* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D020097:Natriuretic Peptide, Brain / Q000097:blood; D011446:Prospective Studies; D012042:Registries; D051437:Renal Insufficiency / Q000097:blood / Q000401:mortality* / Q000503:physiopathology; D015996:Survival Rate; D014655:Vascular Resistance
chemical_list: D020097:Natriuretic Peptide, Brain; D002248:Carbon Monoxide