title: Pulmonary hypertension is associated with higher mortality in cystic fibrosis patients awaiting lung transplantation.
abstract: OBJECTIVE
  Pulmonary hypertension (PH) is frequently found in patients with advanced parenchymal lung diseases. In advanced stages, cystic fibrosis (CF) patients can develop PH and eventually cor pulmonale. Little is known about the prevalence of PH in CF patients and its impact on outcome.
  
  
  METHODS
  We retrospectively studied a large cohort of CF patients evaluated for lung transplantation between 1995 and 2010. All the patients underwent right heart catheterization as part of the evaluation. We included 179 unique consecutive adult CF patients. Age was 24±9 years and 45.8% were women.
  
  
  RESULTS
  Eighty-seven patients were transplanted (48.6%) and 65 died (36.3%) while waiting for LT. By right heart catheterization, 38.5% of the patients had PH (mean ≥25 mm Hg). PaCO(2) (P=0.045) and forced vital capacity (P=0.023) were independent predictors of PH in CF patients. The median survival (free of lung transplantation) was 13.4 months. After adjusting for several covariates, the presence of PH significantly increased mortality (hazard ratio, HR) (P<0.001). Pulmonary vascular resistance was associated with mortality (P=0.03). When both PH and PVR were included in the model, only PH predicted mortality.
  
  
  CONCLUSIONS
  Pulmonary hypertension of mild degree is frequently found in CF patients with advanced lung disease and its presence significantly worsens survival.
authors.affiliation: Department of Thoracic Surgery, La Sapienza University of Rome, Rome, Italy. federico.venuta@uniroma1.it
authors.forename: F
authors.initials: F
authors.lastname: Venuta
authors.forename: A R
authors.initials: AR
authors.lastname: Tonelli
authors.forename: M
authors.initials: M
authors.lastname: Anile
authors.forename: D
authors.initials: D
authors.lastname: Diso
authors.forename: T
authors.initials: T
authors.lastname: De Giacomo
authors.forename: F
authors.initials: F
authors.lastname: Ruberto
authors.forename: E
authors.initials: E
authors.lastname: Russo
authors.forename: M
authors.initials: M
authors.lastname: Rolla
authors.forename: S
authors.initials: S
authors.lastname: Quattrucci
authors.forename: E A
authors.initials: EA
authors.lastname: Rendina
authors.forename: N
authors.initials: N
authors.lastname: Li Phd
authors.forename: G F
authors.initials: GF
authors.lastname: Coloni
mesh_terms: D000328:Adult; D003550:Cystic Fibrosis / Q000150:complications / Q000401:mortality* / Q000601:surgery*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D016040:Lung Transplantation; D008297:Male; D015995:Prevalence; D012189:Retrospective Studies; D012307:Risk Factors; D012680:Sensitivity and Specificity; D015996:Survival Rate; D014850:Waiting Lists; D055815:Young Adult