[citation needed] While Ebstein's anomaly is defined as the congenital displacement of the tricuspid valve towards the apex of the right ventricle, it is often associated with other abnormalities.

[citation needed] About 50% of individuals with Ebstein's anomaly have an associated shunt between the right and left atria, either an atrial septal defect or a patent foramen ovale.

[6] About 50% of individuals with Ebstein's anomaly have an accessory pathway with evidence of Wolff-Parkinson-White syndrome, secondary to the atrialized right ventricular tissue.

An echocardiogram is the most common and specific way to diagnose Ebstein’s anomaly because it effectively shows all 4 chambers of the heart, which displays the distance between the hinge point of the septal leaflet of the tricuspid valve and the anterior leaflet of the mitral valved (displacement index) to determine if the value is greater than 8mm/m2.

If atrial fibrillation with pre-excitation occurs, treatment options include procainamide, flecainide, propafenone, dofetilide, and ibutilide, since these medications slow conduction in the accessory pathway causing the tachycardia and should be administered before considering electrical cardioversion.