心血管
词汇介绍
拓展阅读
解析
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。
概述
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(又称Taussig-Bing畸形);VSD邻近2个大动脉开口(双邻近型);ⅤSD远离2个大动脉开口(远离型)。
诊断标准
1. 症状
自幼出现心悸、气短,易患上呼吸道感染,生长发育受限,有发绀及杵状指。
2. 体征
胸骨左缘第2-4肋问隙可闻4/6级以上收缩期杂音,并可触及细震颤。有漏斗部或肺动脉瓣狭窄者,类似法洛四联症。无肺动脉狭窄者,类似大型室间隔缺损伴肺动脉高压症。
3.辅助检查
(1)X线胸片(心脏远达位):心影增大,有肺动脉狭窄者,肺纹理减少;无肺动脉狭窄者,肺纹理增多。
(2)心电网常见有心室肥厚。
(3)超声心动图:主动脉位置前移,主动脉和肺动脉均起源于右心室,室间隔缺损为左心室的唯一出口,二尖瓣和主动脉瓣间被圆锥组织分隔开,无纤维组织连接。
(4)心导管检查左、右心室压力相等,无肺动脉狭窄者,肺动脉压升高,右心事造影主动脉和肺动脉可同时显影。侧位片显示主动脉瓣和肺动脉瓣在同一平面。
治疗原则
1.手术适应证
(1)2岁以下儿童无肺动脉狭窄,伴有肺动脉高压者,可选肺动脉环缩术作为第一期手术以减轻肺动脉高压;而伴有肺动脉狭窄严重者,可做体-肺动脉分流术。目前多选择一期手术修补VSD,即VSD与主动脉瓣建立内隧道。
(2)2岁以上患者,如肺动脉阻力<10 Wood,或肺/体循环压力比<0.85,均做一期根治。
(3)对于肺动脉发育不良,或合并复杂畸形如房室瓣反流、三尖瓣骑跨的病例,多根据具体病理情况采用单心室修复或姑息手术,包括全腔静脉-肺动脉吻合术、上腔静脉-肺动脉端-侧吻合术、肺动脉环缩术等。
2.手术方法
(1)房室关系一致的右心室双出口
①主动脉瓣下室间隔缺损:如无肺动脉狭窄,按巨大室间隔缺损的手术治疗处理。如合并肺动脉狭窄,应进行心室内补片修补建立内隧道;切除漏斗部肥厚肌束,扩大形成流出道,严重者应用带瓣外管道连接有心室与肺动脉。
②肺动脉瓣下室间隔缺损(Taussig - Bing畸形):目前临床上多采用心内补片,将VSD连接至肺动脉,然后进行大动脉调转手术(ASO)。
③双邻近型或远离型室间隔缺损:根治术多需进行心内补片,多数情况下需要植入右心室-肺动脉外管道。
(2)房室关系不一致的有室双出口:无论室间隔缺损的位置如何,以及有无肺动脉狭窄,手术方法均可经有心室闭合室间隔缺损,缝闭肺动脉瓣口或结扎肺动脉近端,然后植入有心室-肺动脉外管道。
动脉转位术后主动脉根部扩张和主动脉瓣返流的进展
发表时间:2019-07-10
影响指数:5.1
作者: Roel L F van der Palen
期刊:Heart
The arterial switch operation (ASO) has been a significant milestone in the evolution of surgery for transposition of the great arteries (TGA) and after its introduction in 1975 has gradually replaced the atrial switch procedure worldwide. Despite excellent late survival with good functional ability, residual problems are increasingly recognised during long-term follow-up and include dilatation of the neo-aortic root and neo-aortic valve regurgitation (AR) that may result in neo-aortic root replacement. It has been reported that the neo-aortic root dilates in more than two-thirds of patients after ASO. However, data on progression of neo-aortic dilatation in adulthood are scarce and controversial. Similar to root dilatation, concerns have risen about the neo-aortic valve function over time and AR has been described as an important cause for reoperation. The purpose of this study was to assess neo-aortic growth, neo-aortic valve function and the need for reoperations on neo-aortic valve and/or root during long-term follow-up for the various morphological subtypes of TGA after ASO and, finally, to identify risk factors for root dilatation and AR.
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