title: Outcome of congenital diaphragmatic hernia with indication for Fontan procedure.
abstract: BACKGROUND
  The aim of this study was to clarify the outcome of patients with cardiovascular malformation (CVM) among those with congenital diaphragmatic hernia (CDH) who are indicated for the Fontan procedure.
  
  
  METHODS
  The subjects included 76 CDH patients with CVM recruited from a national survey of 614 CDH patients. The outcomes were evaluated between two groups divided according to indication for the Fontan procedure. Patients with functional univentricular disease were considered to be candidates for the Fontan procedure.
  
  
  RESULTS
  Sixteen (21.1%) of the 76 patients were candidates for the Fontan procedure, accounting for 2.6% of all 614 patients with CDH. None of these patients, however, underwent the Fontan procedure. Among the 16 patients, the absence of obstruction of the left ventricular outflow tract (LVOTO) was significantly associated with better 90 day survival (71.4%, 5/7, for those without LVOTO vs 0.0%, 0/9, for those with LVOTO, P = 0.0007). After excluding 22 patients with chromosomal and/or genetic abnormalities or syndromes, the 90 day survival rate was significantly better in neonates without than with indication for the Fontan procedure (62.5%, 25/40 vs 28.6%, 4/14, P = 0.0271).
  
  
  CONCLUSIONS
  Patients with indications for the Fontan procedure are rare, and the outcome of patients with LVOTO among those with CDH is especially poor.
authors.affiliation: Department of Pediatric Cardiology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan.
authors.forename: Nobuko
authors.initials: N
authors.lastname: Shiono
authors.forename: Noboru
authors.initials: N
authors.lastname: Inamura
authors.forename: Shigehiro
authors.initials: S
authors.lastname: Takahashi
authors.forename: Kouji
authors.initials: K
authors.lastname: Nagata
authors.forename: Yuji
authors.initials: Y
authors.lastname: Fujino
authors.forename: Masahiro
authors.initials: M
authors.lastname: Hayakawa
authors.forename: Noriaki
authors.initials: N
authors.lastname: Usui
authors.forename: Hiroomi
authors.initials: H
authors.lastname: Okuyama
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Kanamori
authors.forename: Tomoaki
authors.initials: T
authors.lastname: Taguchi
authors.forename: Hisanori
authors.initials: H
authors.lastname: Minakami
mesh_terms: D000015:Abnormalities, Multiple / Q000601:surgery*; D018376:Cardiovascular Abnormalities / Q000150:complications / Q000601:surgery*; D018729:Fontan Procedure; D006352:Heart Ventricles; D065630:Hernias, Diaphragmatic, Congenital / Q000150:complications / Q000601:surgery*; D006801:Humans; D007231:Infant, Newborn; D012189:Retrospective Studies; D016896:Treatment Outcome
keywords: Fontan procedure; cardiovascular malformation; congenital diaphragmatic hernia; national survey