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Double-outlet right ventricle and overriding tricuspid valve reflect disturbances of looping, myocardialization, endocardial cushion differentiation, and apoptosis in TGF-beta(2)-knockout mice.
双出口右心室和覆盖三尖瓣反映了 TGF-β (2) 基因敲除小鼠的循环、心肌化、心内膜垫分化和细胞凋亡的紊乱。

摘要

BACKGROUND:Transforming growth factor-beta(2) (TGF-beta(2)) is a member of a family of growth factors with the potential to modify multiple processes. Mice deficient in the TGF-beta(2) gene die around birth and show a variety of defects of different organs, including the heart.
METHODS AND RESULTS:We studied the hearts of TGF-beta(2)-null mouse embryos from 11.5 to 18.5 days of gestation to analyze the types of defects and determine which processes of cardiac morphogenesis are affected by the absence of TGF-beta(2). Analysis of serial sections revealed malformations of the outflow tract (typically a double-outlet right ventricle) in 87.5%. There was 1 case of common arterial trunk. Abnormal thickening of the semilunar valves was seen in 4.2%. Associated malformations of the atrioventricular (AV) canal were found in 62.5% and were composed of perimembranous inlet ventricular septal defects (37.5%), AV valve thickening (33.3%), overriding tricuspid valve (25.0%), and complete AV septal defects (4.2%). Anomalies of the aorta and its branches were seen in 33.3%. Immunohistochemical staining showed failure of myocardialization of the mesenchyme of the atrial septum and the ventricular outflow tract as well as deficient valve differentiation. Morphometry documented this to be associated with absence of the normal decrease of total endocardial cushion volume in the older stages. Apoptosis in TGF-beta(2)-knockout mice was increased, although regional distribution was normal.
CONCLUSIONS:TGF-beta(2)-knockout mice exhibited characteristic cardiovascular anomalies comparable to malformations seen in the human population.

译文

背景: 转化生长因子-β (2) (TGF-β (2)) 是生长因子家族的一员,具有改变多种过程的潜力。TGF-β (2) 基因缺陷的小鼠在出生后死亡,并显示出包括心脏在内的不同器官的各种缺陷。
方法和结果: 我们研究了 TGF-β (2) 的心脏 -从妊娠 11.5 到 18.5 天的无效小鼠胚胎,以分析缺陷的类型,并确定哪些心脏形态发生过程受到 TGF-β 缺乏的影响 (2)。连续切片的分析显示 87.5% 的流出道畸形 (通常是右心室双出口)。共动脉干 1 例。在 4.2% 中可以看到半月瓣异常增厚。62.5% 的人发现了与房室管 (AV) 相关的畸形,包括膜周部入口室间隔缺损 (37.5%),AV 瓣膜增厚 (33.3%),高于三尖瓣 (25.0%), 和完整的 AV 间隔缺陷 (4.2%)。主动脉及其分支的异常见于 33.3%。免疫组织化学染色显示房间隔和心室流出道的间充质心肌化失败以及瓣膜分化不足。形态测量法记录了这与老阶段总心内膜垫量正常减少的缺失有关。TGF-β (2) 基因敲除小鼠的细胞凋亡增加,尽管区域分布正常。
结论: TGF-β (2) 基因敲除小鼠表现出与人类畸形相当的心血管异常特征。

double-outlet right ventricle

心血管 DORV 疾病
概述  :  

右心室双出口(DORV)是指主动脉和肺动脉完全或几乎完全发自形态右心室,发病率约0.09%,占所有先天性心脏病的1%~5%。其病理特征包括:①主动脉、肺动脉均全部出自解剖右心室;②室间隔缺损为解剖左心室的唯一出口;③两组半月瓣下均有肌性圆锥(双圆锥),主动脉瓣与二尖瓣的纤维连续中断,被肌性圆锥分隔;④两组半月瓣位于同一高度DORⅤ通常根据两大动脉的关系、室间隔缺损的位置和右心室流出道有无梗阻进行分类。根据室间隔缺损(VSD)的位置,DORV分为4类:VSD位于主动脉瓣下;VSD位于肺动脉瓣下

double-outlet

释义   双出口

例句   To evaluate the results of surgical treatment of double-outlet of right ventricle . 

目的探讨右心室双出口的手术治疗效果。

 

right ventricle

释义   [解剖] 右心室;右室

例句   The variations of left atrium, right ventricle and pulmonary arteries were not parelleled. 

左心房、右心室和肺动脉的改变不成比例。


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