总肺静脉回流异常(TAPVR)很少见(约占所有CHD的1%),可以作为单个病变或与其他类型的CHD(例如异位或HLHS)合并发生。TAPVR被定义为异常连接,其中所有肺静脉都不会直接或通过连接到右心房的静脉排入左心房,而是排入右心房。TAPVR可分为四个解剖组(图。32.1):(1)心上(约55%),(2)心脏(约30%),(3)心外(约13%),和(4)混合(非常罕见)。此外,它可以分为两种生理类型:非阻塞和阻塞。胚胎学上,所有肺静脉通常都连接到与左心房相连的肺静脉汇合处。如果未发生此连接,肺静脉汇合处连接到全身静脉。
Total anomalous pulmonary venous return (TAPVR) is rare (accounting for about 1% of all CHD) and can occur as a single lesion or in combination with other types of CHD (such as heterotaxy or HLHS). TAPVR is defined as an abnormal connection where all pulmonary veins do not drain into the left atrium but into the right atrium either directly or through a vein that is connected to the right atrium. TAPVR can be divided into four anatomic groups (Fig. 32.1): (1) supracardiac (about 55%), (2) cardiac (about 30%), (3) infracardiac (about 13%), and (4) mixed (very rare). In addition, it can be divided into two physiological types: nonobstructed and obstructed. Embryologically, all pulmonary veins usually connect to a pulmonary venous confluence that connects to the left atrium. If this connection does not occur, the pulmonary venous confluence connects to a systemic vein instead.