title: Contemporary outcomes after the Fontan procedure: a Pediatric Heart Network multicenter study.
abstract: OBJECTIVE
  We characterized a large cohort of children who had a Fontan procedure, with measures of functional health status, ventricular size and function, exercise capacity, heart rhythm, and brain natriuretic peptide (BNP).
  
  
  BACKGROUND
  The characteristics of contemporary Fontan survivors are not well described.
  
  
  METHODS
  We enrolled 546 children (age 6 to 18 years, mean 11.9 years) and compared them within pre-specified anatomic and procedure subgroups. History and outcome measures were obtained within a 3-month period.
  
  
  RESULTS
  Predominant ventricular morphology was 49% left ventricular (LV), 34% right ventricular (RV), and 19% mixed. Ejection fraction (EF) was normal for 73% of subjects; diastolic function grade was normal for 28%. Child Health Questionnaire mean summary scores were lower than for control subjects; however, over 80% of subjects were in the normal range. Brain natriuretic peptide concentration ranged from <4 to 652 pg/ml (median 13 pg/ml). Mean percent predicted peak O2 consumption was 65% and decreased with age. Ejection fraction and EF Z score were lowest, and semilunar and atrioventricular (AV) valve regurgitation were more prevalent in the RV subgroup. Older age at Fontan was associated with more severe AV valve regurgitation. Most outcomes were not associated with a superior cavopulmonary connection before Fontan.
  
  
  CONCLUSIONS
  Measures of ventricular systolic function and functional health status, although lower on average in the cohort compared with control subjects, were in the majority of subjects within 2 standard deviations of the mean for control subjects. Right ventricular morphology was associated with poorer ventricular and valvular function. Effective strategies to preserve ventricular and valvular function, particularly for patients with RV morphology, are needed.
authors.affiliation: Duke University Medical Center, Durham, North Carolina 27710, USA. ander005@mc.duke.edu
authors.forename: Page A W
authors.initials: PA
authors.lastname: Anderson
authors.forename: Lynn A
authors.initials: LA
authors.lastname: Sleeper
authors.forename: Lynn
authors.initials: L
authors.lastname: Mahony
authors.forename: Steven D
authors.initials: SD
authors.lastname: Colan
authors.forename: Andrew M
authors.initials: AM
authors.lastname: Atz
authors.forename: Roger E
authors.initials: RE
authors.lastname: Breitbart
authors.forename: Welton M
authors.initials: WM
authors.lastname: Gersony
authors.forename: Dianne
authors.initials: D
authors.lastname: Gallagher
authors.forename: Tal
authors.initials: T
authors.lastname: Geva
authors.forename: Renee
authors.initials: R
authors.lastname: Margossian
authors.forename: Brian W
authors.initials: BW
authors.lastname: McCrindle
authors.forename: Stephen
authors.initials: S
authors.lastname: Paridon
authors.forename: Marcy
authors.initials: M
authors.lastname: Schwartz
authors.forename: Mario
authors.initials: M
authors.lastname: Stylianou
authors.forename: Richard V
authors.initials: RV
authors.lastname: Williams
authors.forename: Bernard J
authors.initials: BJ
authors.lastname: Clark
mesh_terms: D000293:Adolescent; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003430:Cross-Sectional Studies; D015444:Exercise / Q000502:physiology; D005260:Female; D018729:Fontan Procedure; D006304:Health Status; D006321:Heart / Q000502:physiology; D006330:Heart Defects, Congenital / Q000097:blood / Q000473:pathology / Q000503:physiopathology / Q000601:surgery*; D006352:Heart Ventricles / Q000473:pathology; D006801:Humans; D008297:Male; D020097:Natriuretic Peptide, Brain / Q000097:blood; D012737:Sex Factors; D016896:Treatment Outcome; D016276:Ventricular Function / Q000502:physiology
chemical_list: D020097:Natriuretic Peptide, Brain