title: Pulmonary hypertension in cystic fibrosis with advanced lung disease.
abstract: BACKGROUND
  The impact of pulmonary hypertension (PH) on survival in cystic fibrosis (CF) remains unclear.
  
  
  OBJECTIVE
  To determine the influence of PH on survival in the CF population.
  
  
  METHODS
  The United Network for Organ Sharing database was queried from 1987 to 2013 to identify first-time lung transplant candidates who were tracked from wait list entry date until death or censoring to determine influence of PH. Using right heart catheterization measurements, mild PH was defined as mean pulmonary artery pressure greater than or equal to 25 mm Hg and severe greater than or equal to 35 mm Hg.
  
  
  RESULTS
  Of 2,781 CF patients, 2,100 were used for univariate analysis, 764 for Kaplan-Meier survival function, 687 for multivariate Cox models, and 576 and 132 for matching on the propensity of mild PH and severe PH, respectively. Univariate Cox analysis found significant differences in survival for mild PH (hazard ratio [HR], 1.747; 95% confidence interval [CI], 1.387-2.201; P < 0.001) and severe PH (HR, 2.299; 95% CI, 1.639-3.225; P < 0.001). Further assessment by multivariate Cox models identified significant risk for death associated with mild PH (HR, 1.757; 95% CI, 1.367-2.258; P < 0.001) and severe PH (HR, 2.284; 95% CI, 1.596-3.268; P < 0.001). Cox regression stratified on matched pairs of PH cases and control subjects confirmed the risk for death for mild PH (HR, 1.919; 95% CI, 1.290-2.85; P = 0.001) and severe PH (HR, 4.167; 95% CI, 1.709-10.157; P = 0.002).
  
  
  CONCLUSIONS
  The manifestation of PH is associated with significantly increased risk for death in CF patients with advanced lung disease.
authors.affiliation: 1 Department of Pediatrics.
authors.forename: Don
authors.initials: D
authors.lastname: Hayes
authors.forename: Joseph D
authors.initials: JD
authors.lastname: Tobias
authors.forename: Heidi M
authors.initials: HM
authors.lastname: Mansour
authors.forename: Stephen
authors.initials: S
authors.lastname: Kirkby
authors.forename: Karen S
authors.initials: KS
authors.lastname: McCoy
authors.forename: Curt J
authors.initials: CJ
authors.lastname: Daniels
authors.forename: Bryan A
authors.initials: BA
authors.lastname: Whitson
mesh_terms: D000293:Adolescent; D000328:Adult; D003550:Cystic Fibrosis / Q000150:complications* / Q000401:mortality / Q000601:surgery; D016208:Databases, Factual; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000401:mortality*; D053208:Kaplan-Meier Estimate; D016040:Lung Transplantation; D008297:Male; D015999:Multivariate Analysis; D057216:Propensity Score; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D015996:Survival Rate; D014481:United States; D055815:Young Adult
keywords: advanced lung disease; cystic fibrosis; pulmonary hypertension; survival