Buber Jonathan, Schwaegler Rebecca Gibbons, Mazor Dray Efrat
Division of Cardiology, Department of Medicine, University of Washington School of Medicine, Seattle, WA, USA.
Leviev Heart Center, Sheba Medical Center, Tel Hashomer, Israel.
Echocardiography. 2019 Jul;36(7):1381-1390. doi: 10.1111/echo.14421. Epub 2019 Jun 27.
Since the surgical palliation of a univentricular congenital heart defect was first described in the early 1970s, thousands of lives were saved by this groundbreaking operation. Although initially described for the palliation of tricuspid valve atresia, the Fontan operation is now utilized for many other univentricular heart defects involving either hypoplastic right or left ventricles, and the number of babies who survive the surgery and the postsurgical hospitalization continues to grow. Echocardiography constitutes the mainstay follow-up imaging modality for patients with a Fontan system. In order to obtain all the needed information from the echocardiographic studies, close familiarity with the native congenital heart defects, the build of the Fontan systems, and the potential long-term complications is required. Detailed studies provide valuable information on the various components of the Fontan circulation such as the function of the single ventricle and the valves, flow in the Glenn and Fontan pathways, and possible sources of cyanosis and shunts. Three-dimensional and transesophageal echocardiogram studies can assist in delineating the Fontan anatomy and to evaluate smaller intracardiac structures.
自20世纪70年代初首次描述单心室先天性心脏缺陷的外科姑息治疗以来，这项开创性手术挽救了数千人的生命。虽然最初是为三尖瓣闭锁的姑息治疗而描述的，但目前Fontan手术也用于许多其他涉及右心室或左心室发育不全的单心室心脏缺陷，术后存活及术后住院的婴儿数量持续增加。超声心动图是Fontan系统患者主要的随访成像方式。为了从超声心动图研究中获取所有所需信息，需要密切熟悉先天性心脏缺陷的原始情况、Fontan系统的结构以及潜在的长期并发症。详细研究可提供有关Fontan循环各个组成部分的有价值信息，如单心室和瓣膜的功能、Glenn和Fontan路径中的血流，以及可能的发绀和分流来源。三维和经食管超声心动图研究有助于描绘Fontan解剖结构并评估较小的心脏内结构。
