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Ebstein 畸形:流行病学分析及不同产前管理方法介绍。

Ebstein's anomaly: epidemiological analysis and presentation of different prenatal management.

机构信息

Medical Faculty, Medical University of Lodz, Lodz, Poland.

Department of Obstetrics and Gynaecology, Polish Mother's Memorial Hospital, Lodz, Poland.

出版信息

J Matern Fetal Neonatal Med. 2022 Sep;35(17):3297-3304. doi: 10.1080/14767058.2020.1818207. Epub 2020 Sep 15.

DOI:10.1080/14767058.2020.1818207
PMID:32933366
Abstract

OBJECTIVES

To present the crucial role of echocardiographic examination in perinatal care and analyze influence of prenatal treatment for neonatal outcome. Furthermore, the attempt to answer the question if there was any relationship between the occurrence of fetal Ebstein's anomaly and environmental risk factors in polish population.

METHODS

Forty-five prenatal diagnoses of Ebstein's anomaly were compiled over the 21-year period (1998‒2018) form our single unit. The analysis included the assessment of maternal parameters (age, past obstetric history, and place of residence) and fetal parameters (sex, gestational age, anatomy, the fetal cardiovascular condition assessed by the CVPS, associated extracardiac anomalies or malformations, prenatal treatment, delivery and follow-up).

RESULTS

The average age of gravida was 29.5 years (± 5.2 years) and gravidae <35 years of age accounted for 80% . There were 43 singleton pregnancies and 2 cases of multiple pregnancy. Ebstein's anomaly was mostly (averagely) diagnosed at 28th week of gestation. Forty-three fetuses had normal karyotypes and two had trisomy 21. Cardiomegaly was present in 91% (41) of fetuses. The average heart area to chest area ratio was 0.56 (± 0.12). In 21 cases, there was only fetal monitoring - echocardiographic examinations and postnatal mortality was 44.4%. In 5 cases, transplacental digoxin treatment was administered and mortality was: 40%. In another 5 cases, only steroid therapy was applied and postnatal mortality was 100%. Steroids and transplacental digoxin treatment were administered in 11 cases and mortality was 63.6%. In 3 last cases transplacental digoxin treatment, steroids and maternal hyperoxygenation therapy were given and mortality was 0%. Cesarean section rate was 49%. Moreover, due to Ebstein's anomaly regional peak of occurrence benzopyrene was deliberated as environmental risk factor.

CONCLUSIONS

Fetal Ebstein's anomaly occurred in our population in healthy young women, expecting their first child and malformation was not related to fetus gender, nor to maternal health condition. Our data can be a new signal for the development of novel treatment strategies in therapy in fetuses with Ebstein's anomaly.

摘要

目的

介绍超声心动图检查在围生期护理中的关键作用,并分析产前治疗对新生儿结局的影响。此外,尝试回答波兰人群中胎儿埃布斯坦畸形的发生与环境危险因素之间是否存在关系。

方法

在 21 年期间(1998 年至 2018 年),我们从一个单一的医疗单位收集了 45 例产前诊断的埃布斯坦畸形病例。分析包括评估母亲的参数(年龄、既往产科病史和居住地)和胎儿的参数(性别、胎龄、解剖结构、通过 CVPS 评估的胎儿心血管状况、相关的心脏外畸形或畸形、产前治疗、分娩和随访)。

结果

孕妇的平均年龄为 29.5 岁(±5.2 岁),年龄小于 35 岁的孕妇占 80%。43 例为单胎妊娠,2 例为多胎妊娠。埃布斯坦畸形的平均诊断孕周为 28 周。43 例胎儿的核型正常,2 例为 21 三体。91%(41 例)的胎儿存在心脏增大。心脏面积与胸腔面积的比值平均为 0.56(±0.12)。21 例仅进行胎儿监测-超声心动图检查,产后死亡率为 44.4%。5 例给予了胎儿地高辛治疗,死亡率为 40%。另外 5 例仅给予了类固醇治疗,产后死亡率为 100%。11 例给予了类固醇和胎儿地高辛治疗,死亡率为 63.6%。3 例最后给予了胎儿地高辛治疗、类固醇和母亲高氧治疗,死亡率为 0%。剖宫产率为 49%。此外,由于埃布斯坦畸形,多环芳烃区域高峰被认为是环境危险因素。

结论

我们人群中的胎儿埃布斯坦畸形发生在健康的年轻女性中,她们正在期待第一个孩子,而畸形与胎儿性别无关,也与母亲的健康状况无关。我们的数据可以为胎儿埃布斯坦畸形治疗的新治疗策略的发展提供新的信号。

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Fetal Ebstein's anomaly: early and late appearance.胎儿埃布斯坦畸形:早、晚期表现。
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Pregnancy outcomes in women with Ebstein's anomaly.埃布斯坦畸形女性的妊娠结局。
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The changing epidemiology of Ebstein's anomaly and its relationship with maternal mental health conditions: a European registry-based study.埃布斯坦畸形的流行病学变化及其与孕产妇心理健康状况的关系:一项基于欧洲登记处的研究。
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