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流出道先天性畸形的形态发生学思考。第1部分:共同动脉干和法洛四联症。

Morphogenetic considerations on congenital malformations of the outflow tract. Part 1: Common arterial trunk and tetralogy of Fallot.

作者信息

Bartelings M M, Gittenberger-de Groot A C

机构信息

Department of Anatomy and Embryology, University of Leiden, The Netherlands.

出版信息

Int J Cardiol. 1991 Aug;32(2):213-30. doi: 10.1016/0167-5273(91)90329-n.

Abstract

On the basis of our recent embryologic work concerning the separation process of the outflow tract, together with our study of the morphology of specimens from the Leiden Collection of malformed hearts, we have reconsidered, in conjunction with the pertinent literature, the morphogenesis of common arterial trunk and tetralogy of Fallot. The constant characteristics of common arterial trunk, namely a common trunk, a common arterial orifice and a ventricular septal defect, can be explained simply by absence of the aorto-pulmonary septum, or its complete failure to contribute to the process of separation. The nature of the variable morphologic features, however, is not always clear. Presence of such variation is not contradictory to the explanation of the main characteristics in this malformation. Tetralogy of Fallot, in contrast, can be considered to result from anterior displacement of the columns of the aorto-pulmonary septum relative to the outlet segment and its proximal boundary, the primary fold. Our findings, and suggestions, diverge considerably from conventional theories concerning the morphogenesis of these malformations.

摘要

基于我们近期有关流出道分离过程的胚胎学研究,以及对来自莱顿畸形心脏标本收藏的形态学研究,我们结合相关文献重新审视了共同动脉干和法洛四联症的形态发生。共同动脉干的恒定特征,即共同干、共同动脉口和室间隔缺损,可简单解释为主动脉肺动脉间隔缺失,或其完全未能参与分离过程。然而,可变形态特征的本质并不总是清晰的。这种变异的存在与该畸形主要特征的解释并不矛盾。相比之下,法洛四联症可被认为是由于主动脉肺动脉间隔嵴相对于流出段及其近端边界(原始襞)向前移位所致。我们的发现和建议与关于这些畸形形态发生的传统理论有很大差异。

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