title: Functional outcome after the Fontan operation: factors influencing late morbidity.
abstract: OBJECTIVE
  The purpose of this study was to describe the functional outcome of a large number of patients after modifications of the Fontan operation and to investigate perioperative risk factors that might influence late functional state.
  
  
  METHODS
  A comprehensive cross-sectional review of the first 500 patients undergoing a Fontan operation at our institution was undertaken. Those surviving with an intact Fontan circulation were reviewed by questionnaire to assess functional status and medication history. Medical records, chest roentgenograms, echocardiograms, cardiac catheterizations, and laboratory investigations were also reviewed to assess postoperative status.
  
  
  RESULTS
  Three hundred sixty-three long-term survivors with an intact Fontan circulation were identified during cross-sectional follow-up. Median age at operation was 5.0 years (range 0.4 to 31 years), and median follow-up was 5.4 years (range 1.7 to 20 years). Most patients (91.1%) were in New York Heart Association class I or II. In a multivariate model, poor (class III or IV) functional state was associated with longer duration of follow-up (p < 0.001), a prior atrial septectomy (p = 0.03), and a prior main pulmonary artery-ascending aorta anastomosis (p = 0.05).
  
  
  CONCLUSIONS
  A poor functional outcome is uncommon after the Fontan operation but becomes more frequent with increasing duration of follow-up.
authors.affiliation: Department of Cardiology, Children's Hospital, Boston, MA 02115, USA.
authors.forename: T L
authors.initials: TL
authors.lastname: Gentles
authors.forename: K
authors.initials: K
authors.lastname: Gauvreau
authors.forename: J E
authors.initials: JE
authors.lastname: Mayer
authors.forename: S B
authors.initials: SB
authors.lastname: Fishberger
authors.forename: J
authors.initials: J
authors.lastname: Burnett
authors.forename: S D
authors.initials: SD
authors.lastname: Colan
authors.forename: J W
authors.initials: JW
authors.lastname: Newburger
authors.forename: G
authors.initials: G
authors.lastname: Wernovsky
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000706:statistics & numerical data; D006304:Health Status; D006330:Heart Defects, Congenital / Q000453:epidemiology / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D015993:Life Tables; D016015:Logistic Models; D008297:Male; D009017:Morbidity; D011183:Postoperative Complications / Q000453:epidemiology*; D012086:Reoperation; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome