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伴有二尖瓣反流的左心发育不全综合征:胎儿预后不良的新指标。

Hypoplastic left heart syndrome with mitral regurgitation: a new marker of poor fetal outcome.

作者信息

Witkowski Sławomir, Respondek-Liberska Maria, Strzelecka Iwona

机构信息

Department of Prenatal Cardiology, Polish Mother's Memorial Hospital, Research Institute in Lodz, Poland.

Medical Faculty, Ludwik Rydygier Collegium Medicum, Poland.

出版信息

J Ultrason. 2025 Apr 23;25(100):20250009. doi: 10.15557/jou.2025.0009. eCollection 2025 Jan.

DOI:10.15557/jou.2025.0009
PMID:40656547
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12246897/
Abstract

AIM

Hypoplastic left heart syndrome is a severe congenital heart defect that may be accompanied by tricuspid and mitral valve regurgitation. The aim of this paper is to identify a new ultrasound marker for evaluating the outcomes of fetuses with hypoplastic left heart syndrome.

MATERIAL AND METHODS

This was a single-center analysis of a group of fetuses who underwent fetal ultrasound examinations at our tertiary center between 2016 and 2023. This study included 80 fetuses with hypoplastic left heart syndrome. The gestational age of the studied fetuses ranged from 16.3 to 39.5 weeks. All anomalies and irregularities accompanying hypoplastic left heart syndrome were detected during the second and third trimesters of pregnancy.

RESULTS

Among fetuses with hypoplastic left heart syndrome with tricuspid regurgitation, the mortality rate was 0% (0/16) and the survival rate was 100% (16/16). In contrast, in fetuses with hypoplastic left heart syndrome with both tricuspid and mitral regurgitation, the mortality rate was 75% (3/4) and the survival rate was 25% (1/4). The incidence of death was significantly higher in the group of fetuses with hypoplastic left heart syndrome with both tricuspid and mitral regurgitation compared to the group with tricuspid regurgitation (Yates's chi-squared test: = 0.003; Fisher's test: = 0.0035).

CONCLUSIONS

The coexistence of hypoplastic left heart syndrome with tricuspid and mitral regurgitation is significantly associated with the death of newborns even when treatment and/or surgery is performed. Therefore, the presence of mitral regurgitation in fetuses with hypoplastic left heart syndrome may serve as an additional ultrasound marker for poor neonatal outcome.

摘要

目的

左心发育不全综合征是一种严重的先天性心脏缺陷,可能伴有三尖瓣和二尖瓣反流。本文旨在确定一种新的超声标志物,用于评估左心发育不全综合征胎儿的预后。

材料与方法

这是一项对2016年至2023年在我们三级医疗中心接受胎儿超声检查的一组胎儿进行的单中心分析。本研究纳入了80例左心发育不全综合征胎儿。所研究胎儿的孕周为16.3至39.5周。在妊娠中期和晚期检测到所有伴随左心发育不全综合征的异常和不规则情况。

结果

在患有三尖瓣反流的左心发育不全综合征胎儿中,死亡率为0%(0/16),存活率为100%(16/16)。相比之下,在患有三尖瓣和二尖瓣反流的左心发育不全综合征胎儿中,死亡率为75%(3/4),存活率为25%(1/4)。与仅有三尖瓣反流的胎儿组相比,患有三尖瓣和二尖瓣反流的左心发育不全综合征胎儿组的死亡发生率显著更高(Yates卡方检验:=0.003;Fisher检验:=0.0035)。

结论

即使进行了治疗和/或手术,左心发育不全综合征合并三尖瓣和二尖瓣反流与新生儿死亡显著相关。因此,左心发育不全综合征胎儿中二尖瓣反流的存在可能作为新生儿预后不良的一个额外超声标志物。

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Interatrial Septum Stenting for Hypoplastic Left Heart Syndrome in Ex Utero Intrapartum Therapy.体外宫内治疗胎儿窘迫性左心发育不良综合征的房间隔支架置入术。
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Prenatal diagnosis: Hypoplastic left heart syndrome in combination with ventriculo-coronary communication.产前诊断:左心发育不全综合征合并心室-冠状动脉交通。
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Which Phenotypes Should We Include in the Hypoplastic Left Heart Syndrome?
哪些表型应包括在左心发育不良综合征中?
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Associations Between Maternal Sociodemographics and Hospital Mortality in Newborns With Prenatally Diagnosed Hypoplastic Left Heart Syndrome.产前诊断为左心发育不全综合征的新生儿中,母亲社会人口统计学因素与医院死亡率之间的关联。
Circulation. 2023 Jul 18;148(3):283-285. doi: 10.1161/CIRCULATIONAHA.123.064476. Epub 2023 Jul 17.
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Ultrasonographic Prenatal Diagnosis: Unveiling the Path to Improved Antenatal Care.超声产前诊断:揭示改善产前护理之路。
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Pregnancy loss in major fetal congenital heart disease: incidence, risk factors and timing.重大胎儿先天性心脏病孕妇流产:发生率、危险因素及时间。
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Tricuspid Valve Regurgitation in Hypoplastic Left Heart Syndrome: Current Insights and Future Perspectives.左心发育不全综合征中的三尖瓣反流:当前见解与未来展望
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Fluid Mechanical Effects of Fetal Aortic Valvuloplasty for Cases of Critical Aortic Stenosis with Evolving Hypoplastic Left Heart Syndrome.胎儿主动脉瓣成形术治疗严重主动脉瓣狭窄伴左心发育不良综合征的血流力学效应。
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