Ebstein anomaly


The tricuspid valve (the valve between the right atrium and the right ventricle) and the right ventricle (RV) are malformed. Usually the septal and posterior valve leaflet are displaced in ventricular direction and the corresponding part of the ventricle is thinned (atrialized). The anterior leaftlet is often large. RV function can be normal or, as in Uhl syndrome (greatly enlarged RV) severely impaired. Particularly serious clinical symptoms can be caused by Ebstein anomaly of the left-sided AV valve (AV valve of the systemic ventricle in L-transposition of the great arteries).

Reconstruction/ Operation

The aim of surgical reconstruction is to achieve adequate tricuspid function and improve the pumping function of the RV by excluding the atrialized chamber.
Mobilization and detachment of the anterior and posterior leaflet, longitudinal plication of the atrialized chamber, refixation of the leaflet with a ring to stabilize the valve annulus.
Raising of the posterior leaflet to the tricuspid valve annulus (oblique plication of atrialized chamber along the tricuspid valve annulus.
Da Silva (cone reconstruction), favored at the DHZB
Detachment of the posterior and anterior leaflet, plication of the true tricuspid valve annulus in the area of the atrialized chamber, rotation and combination of the leaflets to form a cone.
Closure of the tricuspid valve leaving a 4 mm fenestration, atrioseptectomy and BT shunt (in neonatal forms). So-called one-and-a half correction (correction plus Glenn operation): if RV function is impaired, children, teenagers and adults may also require a bidirectional upper cavopulmonary anastomosis (Glenn operation).