Ebstein’s Anomaly

Ebstein’s anomaly of the tricuspid valve is a cardiac malformation characterized by downward displacement of the septal and inferior tricuspid valve (TV) leaflets, redundant anterior leaflets with a sail-like morphology, dilation of the true right atrioventricular annulus, TV regurgitation, and dilation of the right atrium and ventricle. The wide variety of anatomic and pathophysiologic presentations of Ebstein’s anomaly has made it difficult to achieve uniform results with surgical repair, resulting in the development of many different surgical techniques for its repair. In 1993, Da Silva et al. developed a surgical technique involving cone reconstruction of the TV. This operation aims to undo most of the anatomic TV defects that occurred during embryologic development and to create a cone-like structure from all available leaflet tissue. The result mimics normal TV anatomy, which is an improvement compared to previously described procedures that result in a monocusp valve coaptation with the ventricular septum. In this chapter, we review the surgical maneuvers that we have used to obtain the best functional TV in cases with several anatomic variations of Ebstein’s anomaly. The cone procedure for reconstruction for Ebstein’s anomaly can be performed with low mortality and morbidity. This tricuspid valve repair is effective and durable for the majority of patients.