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Outcome of congenital diaphragmatic hernia with indication for Fontan procedure
Author(s) -
Shiono Nobuko,
Inamura Noboru,
Takahashi Shigehiro,
Nagata Kouji,
Fujino Yuji,
Hayakawa Masahiro,
Usui Noriaki,
Okuyama Hiroomi,
Kanamori Yutaka,
Taguchi Tomoaki,
Minakami Hisanori
Publication year - 2014
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/ped.12322
Subject(s) - medicine , congenital diaphragmatic hernia , fontan procedure , ventricular outflow tract obstruction , surgery , diaphragmatic breathing , heart disease , cardiology , fetus , pregnancy , mitral valve , genetics , alternative medicine , pathology , biology
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 F ontan 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 F ontan 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 F ontan 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.