title: Lung function and aerobic capacity in adult patients following modified Fontan procedure.
abstract: OBJECTIVE
  To examine cardiopulmonary performance in 52 adult patients with a Fontan circulation.
  
  
  METHODS
  Retrospective cohort study. Values of maximum oxygen uptake (VO(2)max), maximum heart rate (HRmax), forced vital capacity (FVC), and forced expiratory volume in one second (FEV(1)) were compared with predictive values for different age groups. Patients were further subdivided into those with a pulmonary artery connection (RA-PA) or right atrium to right ventricle conduit (RA-RV).
  
  
  RESULTS
  At late follow up (median 10 years, range 1 to 26 years), patients with Fontan circulation had greatly diminished VO(2)max, HRmax, FVC, and FEV(1) compared with predicted values. Early age at surgery had a positive impact on aerobic capacity. The FEV(1):FVC ratio indicated restrictive lung function. No differences were found with respect to any variable between patients with RA-PA connections and those with RA-RV connections.
  
  
  CONCLUSIONS
  Patients with a Fontan circulation have greatly diminished values of aerobic capacity and a restrictive pattern of lung function. Patients with an early surgical procedure obtained higher values of VO(2)max. The theoretical benefits of including the right ventricle in a Fontan circulation were not apparent.
authors.affiliation: Toronto General Hospital (University Health Network), University of Toronto Congenital Cardiac Centre for Adults, 200 Elizabeth Street 12th-215 EN, Toronto, Ontario M5G 2C4, Canada.
authors.forename: P M
authors.initials: PM
authors.lastname: Fredriksen
authors.forename: J
authors.initials: J
authors.lastname: Therrien
authors.forename: G
authors.initials: G
authors.lastname: Veldtman
authors.forename: M A
authors.initials: MA
authors.lastname: Warsi
authors.forename: P
authors.initials: P
authors.lastname: Liu
authors.forename: S
authors.initials: S
authors.lastname: Siu
authors.forename: W
authors.initials: W
authors.lastname: Williams
authors.forename: J
authors.initials: J
authors.lastname: Granton
authors.forename: G
authors.initials: G
authors.lastname: Webb
mesh_terms: D000328:Adult; D015331:Cohort Studies; D005080:Exercise Test; D017079:Exercise Tolerance; D005260:Female; D018729:Fontan Procedure; D005541:Forced Expiratory Volume; D006330:Heart Defects, Congenital / Q000503:physiopathology* / Q000601:surgery; D006339:Heart Rate; D006801:Humans; D016014:Linear Models; D008297:Male; D010101:Oxygen Consumption; D012189:Retrospective Studies; D013147:Spirometry; D014797:Vital Capacity