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左、右心发育不全综合征中心室的冠状动脉及心内膜纤维弹性组织增生症。

Coronary and endocardial fibroelastosis of the ventricles in the hypoplastic left and right heart syndromes.

作者信息

Essed C E, Klein H W, Krediet P, Vorst E J

出版信息

Virchows Arch A Pathol Anat Histol. 1975 Oct 20;368(2):87-97. doi: 10.1007/BF00432410.

DOI:10.1007/BF00432410
PMID:810950
Abstract

In an autopsy material of 29 cases of the hypoplastic left heart syndrome coronary fibroelastosis was found in 1 case, endocardial fibroelastosis in 8 cases. Figures for 10 cases of the hypoplastic right heart syndrome were 6 cases of coronary fibroelastosis and 1 case of endocardial fibroelastosis. Age ranged from stillborn up to 11-1/2 months. Coronary and endocardial fibroelastosis seemed to be mutually exclusive localizations of congenital fibroelastosis since in our material they did not occur together in the same hearts. In hypoplastic right hearts coronary fibroelastosis was either restricted to the right coronary artery (right circumflex and posterior interventricular branch), or it was found also in the left coronary artery (anterior interventricular branch), with the most serve affections always being situated in the right one. In the only case of coronary fibroelastosis among the hypoplastic left hearts the condition was limited to the anterior interventricular branch of the left coronary artery which communicated with the hypoplastic left ventricle by a fistula. Coronary fibroelastosis was exclusively found in branches supplying the hypoplastic right ventricle and/or in a branch connected by a fistula to the hypoplastic left or right ventricle. Endocardial fibroelastosis was generally found in hypoplastic left ventricles with either no outflow or with severe outflow obstruction. A theory concerning the aetiology of both coronary and endocardial fibroelastosis of the hypoplastic ventricles is proposed. It is argued that development of fibroelastosis may in both localizations be caused or favoured by the coincidence of two factors: abnormal haemodynamic conditions and poor oxygenation of blood and tissues. Observations made in a reference material of 35 hypoplastic left and 24 hypoplastic right hearts were in accordance with this view.

摘要

在29例左心发育不全综合征的尸检材料中,发现1例冠状动脉纤维弹性组织增生症,8例心内膜纤维弹性组织增生症。10例右心发育不全综合征的情况是,6例冠状动脉纤维弹性组织增生症,1例心内膜纤维弹性组织增生症。年龄范围从死产至11个半月。冠状动脉和心内膜纤维弹性组织增生症似乎是先天性纤维弹性组织增生症相互排斥的定位,因为在我们的材料中,它们不会在同一心脏中同时出现。在右心发育不全的情况下,冠状动脉纤维弹性组织增生症要么局限于右冠状动脉(右回旋支和后室间支),要么也见于左冠状动脉(前室间支),最严重的病变总是位于右侧。在左心发育不全中唯一的冠状动脉纤维弹性组织增生症病例中,病变局限于左冠状动脉的前室间支,该支通过瘘管与发育不全的左心室相通。冠状动脉纤维弹性组织增生症仅见于供应发育不全的右心室的分支和/或通过瘘管与发育不全的左或右心室相连的分支。心内膜纤维弹性组织增生症一般见于无流出道或有严重流出道梗阻的发育不全的左心室。提出了一个关于发育不全心室的冠状动脉和心内膜纤维弹性组织增生症病因的理论。有人认为,纤维弹性组织增生症在这两种定位中的发生可能是由两个因素的巧合引起或促进的:异常的血流动力学状况以及血液和组织的氧合不足。在35例左心发育不全和24例右心发育不全的参考材料中的观察结果与这一观点一致。

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本文引用的文献

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Anoxia as a cause of endocardial fibroelastosis in infancy.缺氧作为婴儿期心内膜弹力纤维增生症的一个病因。
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