Ebstein畸形的外科治疗.pptxVIP

Ebstein畸形的外科治疗第1页/共51页 Ebstein畸形是罕见复杂的心脏先天畸形发生率1:40,000-200,000先天性心脏病中: 1%疾病谱宽： 轻型 无症状 重症 新生儿期死亡率极高 手术死亡率高Wilhelm Ebstein 1866 年首先描述形态Helen Taussig 1950 年描述临床特点第2页/共51页 解剖学特点Displacement of the septal and posterior leaflets of the TV toward the apex of the RV.Although the anterior leaflet is attached at the appropriate level of the tricuspid annulus, it is larger than normal and may have multiple chordal attachments to the ventricular wall.第3页/共51页 3. The segment of the RV from the level of the true tricuspid annulus to the level of attachment of the septal and posterior leaflets is unusually thin and dysplastic. The tricuspid annulus and the RA are extremely dilated.4. The cavity of the functional RV is reduced in size, usually lacks an inlet chamber, and has a small trabecular component. 第4页/共51页 5. The infundibulum is often obstructed by the redundant tissue of the anterior leaflet as well as by the chordal attachments of the anterior leaflet to the infundibulum.第5页/共51页 临床分型（分级）type A: the volume of the true RV is adequate.type B：there is a large atrialized component of the RV, but the anterior leaflet moves freely.type C: the anterior leaflet is severely restricted in its movement and may cause signficant obstruction of the RVOT.type D: there is almost complete atrialization of the ventricle with the exception of a small infundibular component.The only communication between the atrialized ventricle and the infundibulum is through the anteroseptal commissure of the tricuspid valve.第6页/共51页 超声评估分级面积比值＝右房＋房化右室/功能右室＋左心房室 心脏舒张期四腔心轴面 1级：＝0.5 2级：0.6-1.0 3级：1.1-1.5 4级： 1.5第7页/共51页 病理生理特点：1. 三尖瓣关闭不全 右房明显扩大，卵圆孔右向左分流，右室扩大2. 右室功能不良 有效收缩部分减少，心室膨胀3. 肺动脉发育不良 三尖瓣前叶、乳头肌阻挡，生理性PAA4. 左室受压，呈“夹心饼”，功能受限5. 可伴有室上性或室性心律 第8页/共51页 临床表现：容易疲劳 ，活动后呼吸困难、心悸，紫绀Giuli