Deficiency of the vestibular spine in atrioventricular septal defects in human fetuses with Down syndrome
Deficiency of the vestibular spine in atrioventricular septal defects in human fetuses with Down syndrome
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DOI：
10.1016/s0002-9149(02)03106-5
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发表时间：
2003-01-15
影响因子：
2.8
通讯作者：
Gittenberger-de Groot, AC
中科院分区：
文献类型：
--
作者：
Blom, NA;Ottenkamp, J;Gittenberger-de Groot, AC
关于唐氏综合征房室间隔缺损（AVSD）的形态发生的数据缺乏支持唐氏综合征心脏关键区域的分子研究。因此，我们使用三维图像重建和免疫组织化学标记研究了21三体的人类胚胎和胎儿中完全AVSD的发育。对8例AVSD三体心脏和10例正常心脏进行了检查，孕5 ~ 16周。在AVSD中，肌性隔膜和静脉瓣膜发育。正常情况下，未融合的内膜垫的大小和组织学也正常。然而，位于背侧系膜的心外间充质（前庭棘）的质量减少，并且不沿肺总静脉的右壁向腹侧沿着。因此，第一肌间隔和右肺嵴被视为两个独立的间隔，连接到下内膜垫。肌性第一隔和上方融合的静脉瓣（spurium隔）会聚，并被间充质的小边缘覆盖，形成持续存在的第一口的顶部，并连接到垫子和减少的前庭棘。在7周时，AVSD中的心室间隔与正常心脏发育5至6周相当。在后期，假隔形成假隔的前上利姆布斯，间充质帽成为连接桥接小叶的桥接腱。因此，来自背侧系膜的前庭棘扩张减少似乎在唐氏综合征AVSD的发展中起重要作用。(C)2003年，Excerpta Medica，Inc.
Data on the morphogenesis of atrioventricular septal defect (AVSD) in Down syndrome are lacking to support molecular studies on Down syndrome heart critical region. Therefore, we studied the development of complete AVSD in human embryos and fetuses with trisomy 21 using 3-dimensional graphic reconstructions and immunohistochemical markers. Eight trisomic hearts with AVSD and 10 normal hearts, ranging from 5 to 16 weeks' gestation, were examined. In AVSD, the muscular septum primum and venous valves develop. normally, and the size and histology of the nonfused endocardial cushions also appear normal. However, the mass of extracardiac mesenchyme (vestibular spine), located at the dorsal mesocardium, is reduced and does not protrude ventrally along the right wall of the common pulmonary vein. As a result of this, the muscular septum primum and the right pulmonary ridge are seen as 2 separate septa that attach to the inferior endocardial cushion. Both the muscular septum primum and the superiorly fused venous valves (septum spurium) converge and are capped by a small rim of mesenchyme, which forms the roof of the persisting ostium primum and connects to cushions and the reduced vestibular spine. At 7 weeks, ventricular septation in AVSD is comparable to 5 to 6 weeks of normal cardiac development. At later stages, the septum spurium forms the anterosuperior limbus of the septum secundum and the mesenchymal cap becomes the bridging tendon that connects the bridging leaflets. Therefore, reduced expansion of the vestibular spine derived from the dorsal mesocardium appears to play an important role in the development of AVSD in Down syndrome. (C) 2003 by Excerpta Medica, Inc.