Identification and Morphogenesis of Vestibular Atrial Septal Defects.
Identification and Morphogenesis of Vestibular Atrial Septal Defects.
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DOI：
10.3390/jcdd7030035
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发表时间：
2020-09-10
影响因子：
2.4
通讯作者：
Spicer DE
中科院分区：
文献类型：
--
作者：
Loomba RS;Tretter JT;Mohun TJ;Anderson RH;Kramer S;Spicer DE
背景：前庭性房间隔缺损是一种位于房间隔前下方的房间交通。反映了发育过程中前庭棘和间充质帽的肌化不足，或发育中的前下间隔成分中的过度凋亡，前庭缺损代表了一种罕见的真正的房间隔缺损。我们审查了来自三个不同档案的尸检标本，以确定此类前庭缺陷的频率及其相关的心脏发现，并提供了来自发育中小鼠心脏的额外分析，以说明其潜在的形态发生。材料与方法：对Farouk S. Ann和Robert H. Idriss心脏登记研究。伊利诺伊州芝加哥的Lurie儿童医院，佛罗里达盖恩斯维尔的佛罗里达大学的货车Mierop档案馆，以及佛罗里达彼得堡的约翰霍普金斯儿童心脏研究所的档案馆，确定了所有表现出前庭房间隔缺损的患者，沿着相关的心内畸形。然后，我们评估了存在这种缺陷的潜在机制，基于使用episcopic显微镜技术制备的450个发育中小鼠心脏数据集的评估。结果：共分析了2100例标本。其中，68例（3%）被发现有前庭房间隔缺损。在胚胎数据15.5时处死的10个发育中的小鼠胚胎中发现了类似的缺陷，在该阶段，房间隔的前下部分通常正常形成。结论：前庭缺损是一种位于卵圆窝前下缘肌层的真性间隔缺损。我们对尸检心脏的回顾性研究表明，这种缺陷可能比以前认为的更常见。提高对缺损位置的认识应优化其未来的临床识别。我们认为，缺陷的存在是因为失败，在胚胎发育过程中，工会的组件，绑定的主要房间隔的前缘房室交界处，无论是因为肌肉化不足或过度凋亡。
Background: The vestibular atrial septal defect is an interatrial communication located in the antero-inferior portion of the atrial septum. Reflecting either inadequate muscularization of the vestibular spine and mesenchymal cap during development, or excessive apoptosis within the developing antero-inferior septal component, the vestibular defect represents an infrequently recognized true deficiency of the atrial septum. We reviewed necropsy specimens from three separate archives to establish the frequency of such vestibular defects and their associated cardiac findings, providing additional analysis from developing mouse hearts to illustrate their potential morphogenesis. Materials and methods: We analyzed the hearts in the Farouk S. Idriss Cardiac Registry at Ann and Robert H. Lurie Children’s Hospital in Chicago, IL, the Van Mierop Archive at the University of Florida in Gainesville, Florida, and the archive at Johns Hopkins All Children’s Heart Institute in St. Petersburg, Florida, identifying all those exhibiting a vestibular atrial septal defect, along with the associated intracardiac malformations. We then assessed potential mechanisms for the existence of such defects, based on the assessment of 450 datasets of developing mouse hearts prepared using the technique of episcopic microscopy. Results: We analyzed a total of 2100 specimens. Of these, 68 (3%) were found to have a vestibular atrial septal defect. Comparable defects were identified in 10 developing mouse embryos sacrificed at embryonic data 15.5, by which stage the antero-inferior component of the atrial septum is usually normally formed. Conclusion: The vestibular defect is a true septal defect located in the muscular antero-inferior rim of the oval fossa. Our retrospective review of autopsied hearts suggests that the defect may be more common than previously thought. Increased awareness of the location of the defect should optimize its future clinical identification. We suggest that the defect exists because of failure, during embryonic development, of union of the components that bind the leading edge of the primary atrial septum to the atrioventricular junctions, either because of inadequate muscularisation or excessive apoptosis.
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影响因子：
1
作者：
Sharratt, GP;Webb, S;Anderson, RH
通讯作者：
Anderson, RH
影响因子：
2.8
作者：
Blom, NA;Ottenkamp, J;Gittenberger-de Groot, AC
通讯作者：
Gittenberger-de Groot, AC
影响因子：
4
作者：
Mohun, Timothy J.;Weninger, Wolfgang J.
通讯作者：
Weninger, Wolfgang J.
影响因子：
20.1
作者：
Webb, S;Brown, NA;Anderson, RH
通讯作者：
Anderson, RH
影响因子：
1
作者：
Fukuda, T;Kashima, I;Yoshiba, S
通讯作者：
Yoshiba, S