Ruano R, Javadian P, Kailin J A, Maskatia S A, Shamshirsaz A A, Cass D L, Zamora I J, Sangi-Haghpeykar H, Lee T C, Ayres N A, Mehollin-Ray A, Cassady C I, Fernandes C, Welty S, Belfort M A, Olutoye O O
Department of Obstetrics and Gynecology, Texas Children's Fetal Center and Baylor College of Medicine, Houston, TX, USA.
Department of Pediatrics, Division of Cardiology, Texas Children's Fetal Center and Baylor College of Medicine, Houston, TX, USA.
Ultrasound Obstet Gynecol. 2015 Jun;45(6):683-8. doi: 10.1002/uog.14648. Epub 2015 May 4.
To evaluate the impact of the presence of a congenital heart anomaly (CHA) and its potential contribution to morbidity and mortality in infants with congenital diaphragmatic hernia (CDH).
In this retrospective cohort study, prenatal and postnatal data of all newborns diagnosed with CDH between January 2004 and December 2012 in a single center were reviewed. Cases were classified into two groups: those with 'isolated' CDH and those with both CDH and CHA. Patients with CHA were further subclassified into those with a major or minor CHA based on the Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1), and the Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery (STS-EACTS) scoring systems. Patients with associated non-cardiac anomalies, including 'syndromic cases', were excluded from the analysis. Primary and secondary outcomes were survival up to 1 year of age and a need for extracorporeal membrane oxygenation (ECMO), respectively.
Of the 180 infants with CDH, 41 were excluded because of the presence of non-cardiac associated anomalies, 118 had isolated CDH and 21 had CDH with CHA (16 with minor and five with major CHA). Receiver-operating characteristics curve analysis demonstrated that the best cut-off for survival was when the score for CHA was ≤ 2 for both RACHS-1 (area under the curve (AUC), 0.74 (P = 0.04); sensitivity, 80.0%; specificity, 87.5%) and STS-EACTS (AUC, 0.83 (P = 0.03); sensitivity, 100%; specificity, 87.5%). Survival rate at 1 year was significantly lower in those with CHD and a major CHA (40.0%; P = 0.04) than in those with isolated CDH (77.1%) and those with CDH and a minor CHA (81.3%). We found no significant differences among the groups with regard to the need for ECMO.
In general, a milder form of CHA does not appear to have a negative impact on the survival of infants with CDH. However, mortality appears to be significantly higher in infants with CDH and a major form of CHA. The scoring systems appear to be useful as predictors for classifying the effects of CHA in this population of patients.
评估先天性心脏异常（CHA）的存在对先天性膈疝（CDH）婴儿发病率和死亡率的影响及其潜在作用。
在这项回顾性队列研究中，对2004年1月至2012年12月在单一中心诊断为CDH的所有新生儿的产前和产后数据进行了回顾。病例分为两组：“单纯性”CDH组和CDH合并CHA组。根据先天性心脏手术风险调整-1（RACHS-1）和胸外科医师协会-欧洲心胸外科协会（STS-EACTS）评分系统，CHA患者进一步分为主要CHA或次要CHA患者。分析排除了伴有非心脏异常的患者，包括“综合征病例”。主要和次要结局分别为1岁时的生存率和体外膜肺氧合（ECMO）需求。
180例CDH婴儿中，41例因存在非心脏相关异常被排除，118例为单纯性CDH，21例为CDH合并CHA（16例为次要CHA，5例为主要CHA）。受试者工作特征曲线分析表明，对于生存率而言，RACHS-1评分系统中CHA评分≤2时（曲线下面积（AUC），0.74（P = 0.04）；灵敏度，80.0%；特异性，87.5%）以及STS-EACTS评分系统中CHA评分≤2时（AUC，0.83（P = 0.03）；灵敏度，100%；特异性，87.5%）是最佳截断值。CHD合并主要CHA患者1岁时的生存率（40.0%；P = 0.04）显著低于单纯性CDH患者（77.1%）和CDH合并次要CHA患者（81.3%）。我们发现各组在ECMO需求方面无显著差异。
一般来说，较轻形式的CHA似乎对CDH婴儿的生存没有负面影响。然而，CDH合并主要形式CHA的婴儿死亡率似乎显著更高。评分系统似乎可作为预测指标，用于对该患者群体中CHA的影响进行分类。
