Ruttenstock Elke, Wright Naomi, Barrena S, Krickhahn Annika, Castellani Christoph, Desai Ashish P, Rintala Risto, Tovar Juan, Till Holger, Zani Augusto, Saxena Amulya, Davenport Mark
Department of Pediatric and Adolescents Surgery, Medical University of Graz, Graz, Austria.
Department of Paediatric Surgery, King's College Hospital, London, United Kingdom.
Eur J Pediatr Surg. 2015 Feb;25(1):3-8. doi: 10.1055/s-0034-1393960. Epub 2015 Jan 5.
Severe lung hypoplasia and persistent pulmonary hypertension are the main determining factors of survival in infants with congenital diaphragmatic hernia (CDH). The oxygenation index (ratio of delivered oxygen and its arterial level) closely reflects lung function. Single-institution studies have reported that best oxygenation index on day 1 of life (BOI-d1) is the most reliable postnatal predictor of survival in CDH. The aim of this study was to evaluate the predictive value of BOI-d1 in four disparate high volume centers in Europe.
A retrospective, multicenter study of infants with CDH born between 2000 and 2009 in four European tertiary institutions was conducted. Ethical approval was obtained from institutional review boards. Centers no. 1 and. 4 offered extracorporeal membrane oxygenation (ECMO), whereas center no. 3 offered fetal endoluminal tracheal occlusion (FETO) in fetuses defined as poor prognosis (lung-to-head ratio [LHR]≤ 1.0 and "liver-up" position). Prenatal LHR and perinatal variables, including gestational age, birth weight, defect side, liver position, BOI-d1, and patch requirement, were analyzed. Receiver operating characteristic curves were used to determine cutoff values for continuous variables. Comparison was made between survivors and nonsurvivors using univariate analysis and logistic regression analysis, p<0.05 was considered significant.
A total of 235 infants (center no. 1, n=29; no. 2, n=64; no. 3, n=113; and no. 4, n=29) were included. One infant required (2%) ECMO and 66 (28%) had FETO. LHR was available in 83 patients (36%). Overall survival (discharge from hospital) and 28-day survival were 67.6% (n=159) and 72.3% (n=170), respectively. Univariate analysis showed that significant categorical predictors of 28-day survival were liver-down position (p<0.0001), LHR >1 (p=0.003), and primary repair (p=0.02) but not defect side (p=0.83). Area under the receiver operating characteristic (AUROC) curve for continuous variables; gestational age, birth weight, and BOI-d1 were 0.70, 0.68, and 0.88, respectively. AUROC for BOI-d1 (28-day survival) was 0.91 and had sensitivities (73 and 91%) and specificities (92 and 80%) for cutoffs of 40 and 82, respectively.
This multicenter study showed, that except from the defect side, all the prenatal variables studied have predictive value but the most useful is BOI-d1. This is simple to calculate and represents an excellent marker for lung function and a reliable early postnatal predictor of survival.
严重肺发育不全和持续性肺动脉高压是先天性膈疝（CDH）患儿生存的主要决定因素。氧合指数（输送的氧气与其动脉水平的比值）密切反映肺功能。单中心研究报告称，出生第1天的最佳氧合指数（BOI-d1）是CDH患儿出生后最可靠的生存预测指标。本研究的目的是评估BOI-d1在欧洲四个不同的高容量中心的预测价值。
对2000年至2009年在欧洲四家三级医疗机构出生的CDH患儿进行了一项回顾性多中心研究。获得了机构审查委员会的伦理批准。第1和第4中心提供体外膜肺氧合（ECMO），而第3中心对预后不良（肺头比[LHR]≤1.0且“肝脏上移”位置）的胎儿提供胎儿腔内气管阻塞（FETO）。分析了产前LHR和围产期变量，包括胎龄、出生体重、缺损侧、肝脏位置、BOI-d1和补片需求。使用受试者工作特征曲线确定连续变量的临界值。采用单因素分析和逻辑回归分析比较存活者和非存活者，p<0.05被认为具有统计学意义。
共纳入235例婴儿（第1中心，n = 29；第2中心，n = 64；第3中心，n = 113；第4中心，n = 29）。1例婴儿（2%）需要ECMO，66例（28%）接受了FETO。83例患者（36%）有LHR数据。总体生存率（出院）和28天生存率分别为67.6%（n = 159）和72.3%（n = 170）。单因素分析显示，28天生存的显著分类预测因素是肝脏下移位置（p<0.0001）、LHR>1（p = 0.003）和一期修补（p = 0.02），而缺损侧不是（p = 0.83）。连续变量胎龄、出生体重和BOI-d1的受试者工作特征（AUROC）曲线下面积分别为0.70、0.68和0.88。BOI-d1（28天生存）的AUROC为0.91，临界值为40和82时的敏感度分别为73%和91%，特异度分别为92%和80%。
这项多中心研究表明，除缺损侧外，所有研究的产前变量都有预测价值，但最有用的是BOI-d1。它计算简单，是肺功能的优秀标志物，也是出生后早期可靠的生存预测指标。
