Outcome of congenital diaphragmatic hernia with indication for Fontan procedure

Nobuko Shiono, Noboru Inamura, Shigehiro Takahashi, Kouji Nagata, Yuji Fujino, Masahiro Hayakawa, Noriaki Usui, Hiroomi Okuyama, Yutaka Kanamori, Tomoaki Taguchi, Hisanori Minakami

Research output: Contribution to journalArticlepeer-review

2 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)553-558
Number of pages6
JournalPediatrics International
Issue number4
Publication statusPublished - 2014

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health


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