右大动脉门产前诊断的影响:全国多中心的ARCADE队列
Sophie Guillaumont1,2, Marie Vincenti2,3, Fanny Thomas2,4
1Foetal and Paediatric Cardiology Explorations Unit, Saint-Pierre Institute, Palavas-les-Flots, France.
概括
对于隔离的右大动脉 (RAA) 的产前查是可靠的,在诊断中具有很好的一致性. 产后CT扫描可以改善RAA的随访,尽管血管分布可能有所不同.
科学领域:
- 心血管医学 心血管医学
- 产前诊断 在产前诊断
- 儿科手术 儿科手术
背景情况:
- 隔离右大动脉门 (RAA) 是一种罕见的先天性异常.
- 在产前诊断RAA对于产后管理至关重要.
- 了解RAA的表现和结果对于临床实践至关重要.
研究的目的:
- 描述在产前诊断的孤立右大动脉的表现.
- 评估产前RAA诊断对产后结果的影响.
- 评估分离RAA的产前查和产后随访方法的可靠性.
主要方法:
- 在出生前诊断的单独RAA的活产儿的多中心回顾性研究 (2010-2019).
- 对所有包括婴儿在内的婴儿进行一年的产后随访.
- 使用Gwet的AC1系数对产前超声波,产后超声波和CT扫描进行诊断一致性的评估.
主要成果:
- 分析了309例单独的RAA病例;83%的人患有左动脉管.
- 在RAA类型的产前和产后超声波之间有很好的一致性 (AC1=0.97).
- 通过CT扫描对RAA诊断具有良好的一致性 (AC1=0.80),但对于血管分布 (AC1=0.21) 是不良的.
- 18%的婴儿有血管环症状,8%的婴儿需要在第一年内进行大动脉门手术.
- 在4%的病例中发现了相关的遗传异常.
结论:
- 隔离RAA的产前查是可靠的,具有高的诊断准确性.
- 产前和产后诊断之间的差异很少见,但可能发生.
- 脑电图扫描对于改善分离RAA的产后随访和管理非常有价值.
- 早期诊断和监测对于管理孤立RAA的潜在并发症至关重要.
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