EBSTEIN's ANOMALY: A FUNCTIONAL CONCEPT AND SUCCESSFUL DEFINITIVE REPAIR

Increased clinical interest,1,2 stimulated by newer diagnostic techniques,3-6 has made it apparent that Ebstein's anomaly represents a spectrum of right heart abnormalities. In the usual case, the septal and posterior leaflets of the tricuspid valve are not attached to the true annulus, as is the enlarged anterior leaflet, but are displaced distally into the ventricle. The degree of mal-attachment and the structural deformities of the cusps may vary greatly. This valvular derangement divides the ventricle into a proximal thin-walled “atrialized” portion and a distal muscular “outflow tract.”