Ebstein畸形的外科治疗.pptVIP

Ebstein畸形的外科治疗;Ebstein畸形是罕见复杂的心脏先天畸形;解剖学特点;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. ;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.;临床分型（分级）;超声评估分级;病理??理特点：;临床表现：;术前基础治疗：;治疗原则：;外科技术：;三尖瓣成形技术;2.改良Carpentier修复;重症Ebstein畸形的定义;新生儿Ebstein畸形的治疗;Preoperative echo assessment patient No. 1 2 3 4 5 RV dysplasia + + 0 0 + tethered anterior leaflet 0 0 + 0 + Echo score ratio 1.3 0.9 0.8 0.6 1.01 severe TR + + + + + functional pulmonary atresia + + + + +;Cardiac catheterization assessment in one neonates;Operative technique ;The right atrium was reduced in size by removing a segment of the right atrial free wall.; Results; Follow-up;双心室矫治(Knott-Craig CJ. Repair of Ebstein’s anomaly in the symptomatic neonate: an evolution of technique with 7-year follow-up .Ann Thorac Surg 2002:73;1786-93) 8 symptomatic patients 6 neonates ( 2-19d, 2.8-3.2kg ) 1 young infant (2mo, 3.8kg) had undergone a starnes operation elsewhere 1 infant (4mo, 6.4kg);Preoperative assessment;Operative technique;Tricuspid valve repair ( 3 had Danielson-type repairs, 3 had DeVega-type repairs, and 2 had complex repairs) ;2.DeVega-type annu