Shiono Nobuko, Inamura Noboru, Takahashi Shigehiro, Nagata Kouji, Fujino Yuji, Hayakawa Masahiro, Usui Noriaki, Okuyama Hiroomi, Kanamori Yutaka, Taguchi Tomoaki, Minakami Hisanori
Department of Pediatric Cardiology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan.
Pediatr Int. 2014 Aug;56(4):553-8. doi: 10.1111/ped.12322. Epub 2014 May 30.
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.
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.
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).
Patients with indications for the Fontan procedure are rare, and the outcome of patients with LVOTO among those with CDH is especially poor.
本研究的目的是阐明先天性膈疝（CDH）患者中，有心血管畸形（CVM）且适合进行Fontan手术者的预后情况。
研究对象包括从614例CDH患者的全国性调查中招募的76例合并CVM的CDH患者。根据是否适合进行Fontan手术将患者分为两组，并对两组的预后进行评估。功能单心室疾病患者被视为Fontan手术的候选者。
76例患者中有16例（21.1%）适合进行Fontan手术，占所有614例CDH患者的2.6%。然而，这些患者均未接受Fontan手术。在这16例患者中，左心室流出道无梗阻（LVOTO）与90天生存率较高显著相关（无LVOTO者为71.4%，5/7；有LVOTO者为0.0%，0/9；P = 0.0007）。排除22例有染色体和/或基因异常或综合征的患者后，无Fontan手术指征的新生儿90天生存率显著高于有指征者（62.5%，25/40 vs 28.6%，4/14；P = 0.0271）。
适合进行Fontan手术的患者很少见且CDH患者中有LVOTO的患者预后特别差。
