title: Right heart catheterization measuring central hemodynamics in cystic fibrosis during exercise.
abstract: BACKGROUND
  Although pulmonary arterial hypertension (PAH) is a potential co-morbidity in cystic fibrosis (CF), right heart catheterization (RHC) is not commonly performed in this patient population until referral for lung transplantation.
  
  
  METHODS
  An non-randomized observational pilot study was performed after an exercise protocol with an upright stationary bicycle was added to RHC performed in patients with CF undergoing evaluation for lung transplantation (LT).
  
  
  RESULTS
  Twelve consecutive patients with advanced lung disease due to CF referred for LT completed RHC with exercise protocol. Transthoracic echocardiography (TTE) prior to the RHC did not identify evidence of PAH or significant structural abnormalities; right and left ventricular systolic function were normal. non-randomized RHC in this same cohort found 75% (9/12) had PAH with an elevation of the mean pulmonary artery pressure (PAP) at rest with a mean (±SD) of 27.8 ± 4.9 mmHg that significantly increased during exercise to 47.2 ± 5.4 mmHg, p = 0.0025. For the last 6 patients, pulmonary vascular resistance was calculated during exercise, with a significant increase from 3.15 ± 0.3 to 12.8 ± 0.6 Wood Units (p = 0.0313) comparing measurements at rest to exercise.
  
  
  CONCLUSIONS
  RHC at rest and during exercise was safely and effectively performed in patients with CF referred for LT. Furthermore, central hemodynamic measurements found significant worsening of PAH during exercise in a small cohort of CF patients with advanced lung disease.
authors.affiliation: Department of Pediatrics, The Ohio State University College of Medicine, Nationwide Children's Hospital, Columbus, OH 43205, USA. hayes.705@osu.edu
authors.forename: Don
authors.initials: D
authors.lastname: Hayes
authors.forename: Curt J
authors.initials: CJ
authors.lastname: Daniels
authors.forename: Heidi M
authors.initials: HM
authors.lastname: Mansour
authors.forename: Benjamin T
authors.initials: BT
authors.lastname: Kopp
authors.forename: Andrew R
authors.initials: AR
authors.lastname: Yates
authors.forename: Karen S
authors.initials: KS
authors.lastname: McCoy
authors.forename: Alpa V
authors.initials: AV
authors.lastname: Patel
authors.forename: Stephen
authors.initials: S
authors.lastname: Kirkby
mesh_terms: D000293:Adolescent; D000328:Adult; D001794:Blood Pressure / Q000502:physiology*; D006328:Cardiac Catheterization / Q000379:methods*; D003550:Cystic Fibrosis / Q000503:physiopathology*; D015444:Exercise / Q000502:physiology*; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D005541:Forced Expiratory Volume / Q000502:physiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D010865:Pilot Projects; D014655:Vascular Resistance / Q000502:physiology*; D014797:Vital Capacity / Q000502:physiology; D055815:Young Adult
keywords: Cystic fibrosis; Exercise; Pulmonary arterial hypertension; Right heart catheterization