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北美胎儿中心用于识别患有严重先天性膈疝胎儿的观察/预期肺头比及肺总量

Observed/Expected Lung-To-Head Ratio and Total Lung Volumes That Identify Fetuses With Severe Congenital Diaphragmatic Hernia in a North American Fetal Center.

作者信息

Gebb Juliana, Flohr Sabrina, Mathew Leny, Oliver Edward R, Barr Kiersten, Gallagher Taryn, Reynolds Thomas A, Ades Anne, Rintoul Natalie, Wild K Taylor, Partridge Emily, Moldenhauer Julie S, Hedrick Holly L

机构信息

Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.

Division of Pediatric General, Thoracic, and Fetal Surgery, Children's Hospital of Philadelphia, Perelman School of Medicine at University of Pennsylvania, Philadelphia, Pennsylvania, USA.

出版信息

Prenat Diagn. 2025 May;45(5):676-685. doi: 10.1002/pd.6789. Epub 2025 Apr 1.

DOI:10.1002/pd.6789
PMID:40169913
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12054394/
Abstract

OBJECTIVE

To define the ultrasound observed/expected lung-to-head ratio (O/E LHR) and magnetic resonance imaging (MRI) observed/expected total lung volume (O/E TLV) cut-offs associated with survival and lack of extracorporeal membrane oxygenation (ECMO) utilization to determine the most severe cohort that may benefit from fetal intervention.

METHODS

Retrospective review of patients with a prenatal diagnosis of isolated left or right congenital diaphragmatic hernia (L CDH, R CDH) seen and delivered at our level III fetal center from January 2013-July 2023. Data were extracted from our clinical outcome database. Characteristics of survivors and non-survivors were compared for both the L CDH and R CDH groups. For both O/E LHR and O/E TLV, the Youden criteria were then used to determine a good sensitivity and specificity for predicting survival and ECMO utilization for L and R CDH, respectively, in Receiver Operator Characteristic (ROC) curve analysis.

RESULTS

340 patients were included in the study, including 283 (83.2%) with L CDH and 57 (16.8%) with R CDH. The median [interquartile range, IQR] O/E LHR for L and R CDH was 37.9 [28.7-47.3] and 49.0 [40.0-64.5], respectively. The median O/E TLV for L and R CDH was 36.0 [28.0-48.0] and 25.3 [23.6-29.8], respectively. For survival, an O/E LHR of 28.1% and O/E TLV of 34.0% and an O/E LHR of 46.8% and O/E TLV of 17.6% were the best cut-offs for L and R CDH, respectively. For ECMO utilization, an O/E LHR of 32.8% and O/E TLV of 35.3% and an O/E LHR of 47.0% and O/E TLV of 22.0% were the best cut-offs for L and R CDH, respectively.

CONCLUSION

We report the best ultrasound O/E LHR and MRI TLV cut-offs associated with survival and lack of ECMO utilization in our cohort.

摘要

目的

确定超声观察到的/预期的肺头比(O/E LHR)以及磁共振成像(MRI)观察到的/预期的总肺容积(O/E TLV)的临界值,这些临界值与生存及无需使用体外膜肺氧合(ECMO)相关,以确定可能从胎儿干预中获益的最严重队列。

方法

回顾性分析2013年1月至2023年7月在我们三级胎儿中心就诊并分娩的产前诊断为单纯性左或右先天性膈疝(L CDH、R CDH)的患者。数据从我们的临床结局数据库中提取。比较L CDH和R CDH组幸存者和非幸存者的特征。对于O/E LHR和O/E TLV,然后在受试者工作特征(ROC)曲线分析中使用约登标准分别确定预测L CDH和R CDH生存及ECMO使用的良好敏感性和特异性。

结果

340例患者纳入研究,其中283例(83.2%)为L CDH,57例(16.8%)为R CDH。L CDH和R CDH的O/E LHR中位数[四分位间距,IQR]分别为37.9[28.7 - 47.3]和49.0[40.0 - 64.5]。L CDH和R CDH的O/E TLV中位数分别为36.0[28.0 - 48.0]和25.3[23.6 - 29.8]。对于生存,O/E LHR为28.1%且O/E TLV为34.0%以及O/E LHR为46.8%且O/E TLV为17.6%分别是L CDH和R CDH的最佳临界值。对于ECMO使用,O/E LHR为32.8%且O/E TLV为35.3%以及O/E LHR为47.0%且O/E TLV为22.0%分别是L CDH和R CDH的最佳临界值。

结论

我们报告了在我们的队列中与生存及无需使用ECMO相关的最佳超声O/E LHR和MRI TLV临界值。

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本文引用的文献

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A Clinical Outcomes Data Archive for a Comprehensive Fetal Diagnosis and Treatment Center.一个综合性胎儿诊断与治疗中心的临床结果数据存档库。
Fetal Diagn Ther. 2025;52(2):139-147. doi: 10.1159/000541877. Epub 2024 Oct 8.
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Fetoscopic Tracheal Occlusion for Isolated Severe Left Diaphragmatic Hernia: A Systematic Review and Meta-Analysis.胎儿镜下气管闭塞术治疗孤立性严重左侧膈疝:一项系统评价和荟萃分析
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Exome sequencing in fetuses with congenital diaphragmatic hernia in a nationwide cohort.
对全国性队列中先天性膈疝胎儿进行外显子组测序。
Prenat Diagn. 2024 Oct;44(11):1288-1295. doi: 10.1002/pd.6622. Epub 2024 Jun 11.
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Neurodevelopmental Outcomes in Children After Fetoscopic Endoluminal Tracheal Occlusion for Severe Congenital Diaphragmatic Hernia: Results From a Multidisciplinary Clinic.胎儿镜腔内气管阻塞术治疗严重先天性膈疝患儿的神经发育转归:多学科临床研究结果。
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Delivery Room Resuscitation of Infants with Congenital Diaphragmatic Hernia: Lessons Learned through Video Review.先天性膈疝患儿产房复苏:通过视频回顾获得的经验教训
Fetal Diagn Ther. 2025;52(2):124-132. doi: 10.1159/000538536. Epub 2024 Mar 28.
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