title: Survival data and predictors of functional outcome an average of 15 years after the Fontan procedure: the pediatric heart network Fontan cohort.
abstract: OBJECTIVE
  Multicenter longitudinal outcome data for Fontan patients surviving into adulthood are lacking. The aim of this study was to better understand contemporary outcomes in Fontan survivors by collecting follow-up data in a previously well-characterized cohort.
  
  
  METHODS
  Baseline data from the Fontan Cross-Sectional Study (Fontan 1) were previously obtained in 546 Fontan survivors aged 11.9 ± 3.4 years. We assessed current transplant-free survival status in all subjects 6.8 ± 0.4 years after the Fontan 1 study. Anatomic, clinical, and surgical data were collected along with socioeconomic status and access to health care.
  
  
  RESULTS
  Thirty subjects (5%) died or underwent transplantation since Fontan 1. Subjects with both an elevated (>21 pg/mL) brain natriuretic peptide and a low Child Health Questionnaire physical summary score (<44) measured at Fontan 1 were significantly more likely to die or undergo transplant than the remainder, with a hazard ratio of 6.2 (2.9-13.5). Among 516 Fontan survivors, 427 (83%) enrolled in this follow-up study (Fontan 2) at 18.4 ± 3.4 years of age. Although mean scores on functional health status questionnaires were lower than the general population, individual scores were within the normal range in 78% and 88% of subjects for the Child Health Questionnaire physical and psychosocial summary score, and 97% and 91% for the SF-36 physical and mental aggregate score, respectively. Since Fontan surgery, 119 (28%) had additional cardiac surgery; 55% of these (n = 66) in the interim between Fontan 1 and Fontan 2. A catheter intervention occurred in 242 (57%); 32% of these (n = 78) after Fontan 1. Arrhythmia requiring treatment developed in 118 (28%) after Fontan surgery; 58% of these (n = 68) since Fontan 1.
  
  
  CONCLUSIONS
  We found 95% interim transplant-free survival for Fontan survivors over an average of 7 years of follow-up. Continued longitudinal investigation into adulthood is necessary to better understand the determinants of long-term outcomes and to improve functional health status.
authors.affiliation: Department of Pediatrics, Medical University of South Carolina, Charleston, SC, USA.
authors.forename: Andrew M
authors.initials: AM
authors.lastname: Atz
authors.forename: Victor
authors.initials: V
authors.lastname: Zak
authors.forename: Lynn
authors.initials: L
authors.lastname: Mahony
authors.forename: Karen
authors.initials: K
authors.lastname: Uzark
authors.forename: Peter
authors.initials: P
authors.lastname: Shrader
authors.forename: Dianne
authors.initials: D
authors.lastname: Gallagher
authors.forename: Stephen M
authors.initials: SM
authors.lastname: Paridon
authors.forename: Richard V
authors.initials: RV
authors.lastname: Williams
authors.forename: Roger E
authors.initials: RE
authors.lastname: Breitbart
authors.forename: Steven D
authors.initials: SD
authors.lastname: Colan
authors.forename: Jonathan R
authors.initials: JR
authors.lastname: Kaltman
authors.forename: Renee
authors.initials: R
authors.lastname: Margossian
authors.forename: Sara K
authors.initials: SK
authors.lastname: Pasquali
authors.forename: Kerstin
authors.initials: K
authors.lastname: Allen
authors.forename: Wyman W
authors.initials: WW
authors.lastname: Lai
authors.forename: Rosalind
authors.initials: R
authors.lastname: Korsin
authors.forename: Bradley S
authors.initials: BS
authors.lastname: Marino
authors.forename: Nicole
authors.initials: N
authors.lastname: Mirarchi
authors.forename: Brian W
authors.initials: BW
authors.lastname: McCrindle
mesh_terms: D000293:Adolescent; D015415:Biomarkers / Q000097:blood; D002170:Canada; D018572:Disease-Free Survival; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects / Q000401:mortality; D006297:Health Services Accessibility; D006304:Health Status; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D016027:Heart Transplantation; D006801:Humans; D053208:Kaplan-Meier Estimate; D008137:Longitudinal Studies; D008297:Male; D020097:Natriuretic Peptide, Brain / Q000097:blood; D016016:Proportional Hazards Models; D011446:Prospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D012959:Socioeconomic Factors; D011795:Surveys and Questionnaires; D013997:Time Factors; D016896:Treatment Outcome; D014481:United States; D055815:Young Adult
chemical_list: D015415:Biomarkers; D020097:Natriuretic Peptide, Brain
keywords: Congenital; Fontan Procedure; Functional Health Status; Heart Defects; Pediatrics