Contemporary Outcomes in Infants With Congenital Heart Disease and Bochdalek Diaphragmatic Hernia
Contemporary Outcomes in Infants With Congenital Heart Disease and Bochdalek Diaphragmatic Hernia
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DOI：
10.1016/j.athoracsur.2012.07.010
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发表时间：
2013-03-01
影响因子：
4.6
通讯作者：
Kunisaki, Shaun M.
中科院分区：
文献类型：
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作者：
Gray, Brian W.;Fifer, Carlen G.;Kunisaki, Shaun M.
背景15%患有先天性膈疝（CDH）的婴儿出生时就伴有心脏异常。本研究的目的是评估该患者人群的当代结局，并确定院内死亡率的潜在危险因素。回顾性分析1997年至2011年期间在一个儿科三级保健转诊中心治疗的所有先天性心脏病先天性心脏病新生儿的数据。216例CDH患者中有40例（18%）有心脏异常。与无心脏异常的患者相比，该组患者的总生存率显著降低（55% vs 81%; p = 0.001）。根据先天性心脏手术风险调整和胸外科医师协会-欧洲心胸外科协会评分系统（分别为p = 0.86和p = 0.87），基于风险分层的心脏异常类型与死亡率之间无相关性。出生体重在存活者和非存活者之间同样没有差异（分别为2.8 +/- 0.6 kg和2.8 +/- 0.9 kg; p = 0.98）。在死亡者中，体外膜肺氧合的使用有增加的趋势（p = 0.13）。血流动力学稳定的婴儿能够进行后续心脏修复，死亡率较低（p = 0.04）。幸存者有广泛的长期发病率，但大多数有一些神经发育障碍的证据。这个大型的单机构系列研究表明，无论心脏畸形的类型如何，合并CDH和先天性心脏病的婴儿的总体预后都是相当可变的。血流动力学不稳定和需要体外膜氧合与较高的死亡率相关。虽然一些长期存活者有很好的结果，但大多数人患有慢性长期疾病。(Ann Thorac Surg 2013;95：929-34）（C）2013年，胸外科医师协会
Background. Fifteen percent of infants with congenital diaphragmatic hernia (CDH) are born with a coexisting cardiac anomaly. The purpose of this study was to evaluate contemporary outcomes in this patient population and to identify potential risk factors for in-hospital mortality.Methods. Data from all CDH neonates with congenital heart disease managed at a single pediatric tertiary care referral center between 1997 and 2011 were retrospectively analyzed.Results. Forty (18%) of 216 CDH patients had a cardiac anomaly. This group was associated with a significant decrease in overall survival when compared with patients without cardiac anomaly (55% versus 81%; p = 0.001). There was no association between type of cardiac anomaly and mortality based on risk stratification according to the Risk Adjustment for Congenital Heart Surgery and The Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery scoring systems (p = 0.86 and p = 0.87, respectively). Birth weight was similarly no different between survivors and nonsurvivors (2.8 +/- 0.6 kg versus 2.8 +/- 0.9 kg, respectively; p = 0.98). There was a trend toward increased extracorporeal membrane oxygenation use among nonsurvivors (p = 0.13). Infants with hemodynamic stability enabling subsequent cardiac repair were associated with lower mortality (p = 0.04). Survivors had a wide spectrum of long-term morbidity, but most had some evidence of neurodevelopmental impairment.Conclusions. This large single-institution series suggests that the overall prognosis of infants with concomitant CDH and congenital heart disease can be quite variable, regardless of the type of heart anomaly. Hemodynamic instability and need for extracorporeal membrane oxygenation correlate with higher mortality. Although some long-term survivors have excellent outcomes, most suffer from chronic, long-term morbidities. (Ann Thorac Surg 2013;95:929-34) (C) 2013 by The Society of Thoracic Surgeons