Takahashi Shigehiro, Sago Haruhiko, Kanamori Yutaka, Hayakawa Masahiro, Okuyama Hiroomi, Inamura Noboru, Fujino Yuji, Usui Noriaki, Taguchi Tomoaki
Division of Neonatology, Center for Maternal-Fetal and Neonatal Medicine, National Center for Child Health and Development, Tokyo, Japan.
Pediatr Int. 2013 Aug;55(4):492-7. doi: 10.1111/ped.12104.
Congenital diaphragmatic hernia is associated with cardiovascular malformation. Many prognostic factors have been identified for isolated congenital diaphragmatic hernia; however, reports of concurrent congenital diaphragmatic hernia and cardiovascular malformation in infants are limited. This study evaluated congenital diaphragmatic hernia associated with cardiovascular malformation in infants. Factors associated with prognosis for patients were also identified.
This retrospective cohort study was based on a Japanese survey of congenital diaphragmatic hernia patients between 2006 and 2010. Frequency and outcome of cardiovascular malformation among infants with congenital diaphragmatic hernia were examined. Severity of congenital diaphragmatic hernia and cardiovascular malformation were compared as predictors of mortality and morbidity.
Cardiovascular malformation was identified in 76 (12.3%) of 614 infants with congenital diaphragmatic hernia. Mild cardiovascular malformation was detected in 19 (33.9%) and severe cardiovascular malformation in 37 (66.1%). Their overall survival rate at discharge was 46.4%, and the survival rate without morbidity was 23.2%. Mortality and morbidity at discharge were more strongly associated with severity of cardiovascular malformation (adjusted OR 7.69, 95%CI 1.96-30.27; adjusted OR 7.93, 95%CI 1.76-35.79, respectively) than with severity of congenital diaphragmatic hernia.
The prognosis for infants with both congenital diaphragmatic hernia and cardiovascular malformation remains poor. Severity of cardiovascular malformation is a more important predictive factor for mortality and morbidity than severity of congenital diaphragmatic hernia.
先天性膈疝与心血管畸形相关。对于孤立性先天性膈疝，已经确定了许多预后因素；然而，关于婴儿同时患有先天性膈疝和心血管畸形的报道有限。本研究评估了婴儿先天性膈疝合并心血管畸形的情况。还确定了与患者预后相关的因素。
这项回顾性队列研究基于日本对2006年至2010年间先天性膈疝患者的调查。检查了先天性膈疝婴儿中心血管畸形的发生率和结局。比较先天性膈疝和心血管畸形的严重程度，作为死亡率和发病率的预测指标。
在614例先天性膈疝婴儿中，有76例（12.3%）被发现患有心血管畸形。其中19例（33.9%）为轻度心血管畸形，37例（66.1%）为重度心血管畸形。他们出院时的总体生存率为46.4%，无并发症的生存率为23.2%。出院时的死亡率和发病率与心血管畸形严重程度的相关性（校正OR分别为7.69，95%CI 1.96 - 30.27；校正OR为7.93，95%CI 1.76 - 35.79）比与先天性膈疝严重程度的相关性更强。
先天性膈疝合并心血管畸形婴儿的预后仍然很差。心血管畸形的严重程度是比先天性膈疝严重程度更重要的死亡率和发病率预测因素。
