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正常与异常相关的大动脉:我们学到了什么?

Normally and abnormally related great arteries: what have we learned?

作者信息

Praagh Richard Van

机构信息

Children's Hospital Boston, Boston, Massachusetts.

出版信息

World J Pediatr Congenit Heart Surg. 2010 Oct;1(3):364-85. doi: 10.1177/2150135110380239.

Abstract

The conus arteriosus or infundibulum was the site of the major cardiovascular evolutionary and developmental adaptation that made possible air-breathing and permanent land-living for vertebrates, including mammals such as ourselves. The subarterial conal free walls perform an embryonic aortic switch procedure by 35 to 44 days of age in utero, based on growth of the left-sided subpulmonary conal free wall and resorption of the right-sided subaortic conal free wall, i.e., complete right-left asymmetry in the development of the subarterial conal free walls. There is only one way of doing the developmental aortic aortic switch procedure right (one way in situs solitus, and its mirror-image in situs inversus), and there are many ways of doing it wrong, resulting in the conotruncal anomalies. The proximal or apical part of the conus arteriosus, the septal band, was the mother of the right ventricular sinus (the lung pump). The conus transformed the single (systemic) circulation of fish into our double (systemic and pulmonary) circulations. The right ventricle (RV) is only about 36% as old as the left ventricle (LV). Most congenital heart disease involves anomalies of the more recently developed RV, congenital heart disease being the most frequent anomaly in liveborn children - almost 1 percent (0.8%).

摘要

动脉圆锥或漏斗部是主要的心血管进化和发育适应的部位,它使得包括我们人类在内的脊椎动物能够进行空气呼吸并永久生活在陆地上。在子宫内,动脉圆锥下壁在35至44日龄时通过左侧肺动脉圆锥下壁的生长和右侧主动脉圆锥下壁的吸收进行胚胎期主动脉转换过程,即动脉圆锥下壁发育完全的左右不对称。进行发育性主动脉转换过程只有一种正确的方式(在正常位是一种方式,在反位是其镜像方式),而有很多错误的方式,会导致圆锥干畸形。动脉圆锥的近端或顶端部分,即间隔带,是右心室窦(肺泵)的起源。动脉圆锥将鱼类的单一(体循环)循环转变为我们的双重(体循环和肺循环)循环。右心室的发育时间仅约为左心室的36%。大多数先天性心脏病涉及较新发育的右心室的异常,先天性心脏病是活产儿中最常见的异常——几乎为1%(0.8%)。

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