Anderson Robert H, Cook Andrew, Brown Nigel A, Henderson Deborah J, Chaudhry Bill, Mohun Timothy
Division of Pediatric Cardiology, Department of Pediatrics, Medical University of South Carolina, Charleston, South Carolina, United States of America.
Cardiol Young. 2010 Dec;20 Suppl 3:92-9. doi: 10.1017/S1047951110001137.
Although malformations involving the ventricular outflow tracts are often described as conotruncal malformations, there is no consensus as to the lesions included in, or excluded from, this category, reflecting, in part, the current lack of precise definitions of the embryonic truncus and conus. Analysis of development of the outflow tract in terms of proximal, intermediate, and distal components greatly facilitates understanding of the morphology of the aortopulmonary window and aortoventricular tunnels. The aortopulmonary windows reflect failure to close the embryonic aortopulmonary foramen, the space between the distal end of the cushions that divide the lumen of the outflow tract itself and the dorsal wall of the aortic sac. The aortopulmonary tunnels are produced subsequent to abnormal development of the cushions themselves. The distal ends of these cushions excavate to produce the sinuses and leaflets of the arterial valves. The proximal parts of the cushions muscularise to form the subpulmonary infundibulum. The middle part of the cushion mass disappears to provide a tissue plane between the infundibulum and the aortic root. Abnormal formation of this area accounts for the various types of aortoventricular tunnel. In our brief review, we show how the anatomy of these lesions correlates with development of the outflow tract.
尽管涉及心室流出道的畸形通常被描述为圆锥动脉干畸形,但对于该类别中包含或排除的病变尚无共识,这在一定程度上反映了目前对胚胎干和圆锥缺乏精确的定义。根据近端、中间和远端成分分析流出道的发育,极大地有助于理解主肺动脉窗和主心室隧道的形态。主肺动脉窗反映了胚胎主肺动脉孔未能闭合,即分隔流出道本身管腔的垫的远端与主动脉囊后壁之间的间隙。主肺动脉隧道是垫本身发育异常后产生的。这些垫的远端凹陷形成动脉瓣的窦和瓣叶。垫的近端部分肌化形成肺动脉漏斗部。垫团的中间部分消失,在漏斗部和主动脉根部之间提供一个组织平面。该区域的异常形成导致了各种类型的主心室隧道。在我们的简要综述中,我们展示了这些病变的解剖结构与流出道发育的相关性。