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先天性膈疝中的肺动脉高压:产前预测及其对新生儿死亡率的影响。

Pulmonary hypertension in congenital diaphragmatic hernia: Antenatal prediction and impact on neonatal mortality.

机构信息

My FetUZ Fetal Research Center, Department of Development and Regeneration, Cluster Woman and Child, Biomedical Sciences, KU Leuven, Leuven, Belgium.

Clinical Department of Obstetrics and Gynaecology, University Hospitals Leuven, Leuven, Belgium.

出版信息

Prenat Diagn. 2022 Sep;42(10):1303-1311. doi: 10.1002/pd.6207. Epub 2022 Jul 20.

DOI:10.1002/pd.6207
PMID:35801282
Abstract

OBJECTIVE

To determine the prevalence of pulmonary hypertension (PAH) in left-sided congenital diaphragmatic hernia (CDH); how we could predict it; and how PAH contributed to the model for mortality prediction.

STUDY DESIGN

Retrospective analysis in three European centers. The primary outcome was the presence of PAH on postnatal day (d) 1, 7, and at discharge. Studied predictors of PAH were: observed/expected-lung/head-ratio (o/e LHR), liver-herniation, fetoscopic endoluminal tracheal occlusion (FETO), and gestational age (GA) at delivery. The combined effect of pre- and postnatal variables on mortality was modeled by Cox regression.

RESULTS

Of the 197 neonates, 56 (28.4%) died. At d1, 67.5% (133/197) had PAH and 61.9% (101/163) by d7. Overall, 6.4% (9/141) had PAH at discharge. At d1, o/e LHR (odds ratio (OR) 0.96) and FETO (OR 2.99) independently correlated to PAH (areas under the curve [AUC]: 0.74). At d7, PAH significantly correlated only with the use of FETO (OR 3.9; AUC: 0.65). None were significant for PAH at discharge. Combining the occurrence of PAH with antenatal biomarkers improved mortality prediction (p = 0.02), in a model including o/e LHR (HR: 0.94), FETO (HR: 0.35), liver herniation (HR: 16.78), and PAH (HR: 15.95).

CONCLUSIONS

Antenatal prediction of PAH was only moderate. The postnatal occurrence of PAH further increases the risk of death. Whereas this may be used to counsel parents in the postnatal period, our study demonstrates there is a need to find more accurate antenatal predictors for PAH.

摘要

目的

确定左侧先天性膈疝(CDH)中肺动脉高压(PAH)的患病率;我们如何预测它;以及 PAH 如何影响死亡率预测模型。

研究设计

在三个欧洲中心进行回顾性分析。主要结局是在出生后第 1 天(d)、第 7 天和出院时是否存在 PAH。研究的 PAH 预测指标包括:观察/预期肺/头比(o/e LHR)、肝脏疝出、胎儿镜腔内气管阻塞(FETO)和分娩时的胎龄(GA)。通过 Cox 回归对产前和产后变量对死亡率的综合影响进行建模。

结果

197 例新生儿中,56 例(28.4%)死亡。在 d1 时,67.5%(133/197)有 PAH,61.9%(101/163)在 d7 时。总体而言,有 6.4%(9/141)在出院时存在 PAH。在 d1 时,o/e LHR(比值比(OR)0.96)和 FETO(OR 2.99)独立与 PAH 相关(曲线下面积(AUC):0.74)。在 d7 时,PAH 仅与 FETO 的使用显著相关(OR 3.9;AUC:0.65)。在出院时,均无意义。将 PAH 的发生与产前生物标志物相结合,改善了死亡率预测(p=0.02),该模型包括 o/e LHR(HR:0.94)、FETO(HR:0.35)、肝脏疝出(HR:16.78)和 PAH(HR:15.95)。

结论

产前预测 PAH 仅为中等。PAH 的产后发生进一步增加了死亡的风险。虽然这可能用于在产后期间向父母提供咨询,但我们的研究表明,需要找到更准确的产前预测 PAH 的方法。

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