Fig. 52.1
Pathologic specimen of Ebstein anomaly (left) and echocardiographic image (right). The septal leaflet is plastered against the septum (failure of delamination), and the anterior leaflet is markedly enlarged and displaced into the right ventricle and tethered to the free wall of the right ventricle. There is a large atrial septal defect. Arrows on the left panel show thinned wall of right ventricle. Arrow on the right panel shows the hinge point where the septal leaflet is attached to the septum. Abbreviations: RA right atrium, LA left atrium, aRV atrialized portion of the right ventricle, LV left ventricle, RV right ventricle. * defines the enlarged anterior leaflet of the tricuspid valve
An atrial septal defect or stretched patent foramen ovale is present in 50–70 % of cases, and 30 % of cases are associated with a right-sided accessory bypass tract or Wolff-Parkinson-White (WPW) syndrome. Atrial arrhythmias are common in patients with Ebstein anomaly. These can be associated with ventricular preexcitation (WPW) or the dilated right atrium. Accessory conduction pathways should be identified and ablated during a preoperative electrophysiologic study.
The pathophysiology depends on the degree of dysplasia of the tricuspid valve, the degree of tricuspid valve insufficiency, or, in rare cases, tricuspid valve stenosis. In addition, determinants of clinical severity include the severity of right ventricular myopathy; the presence or absence of an interatrial communication; the presence, absence, and degree of right ventricular outflow tract obstruction; and the presence or absence of ventricular preexcitation.
52.3 Clinical Presentation
The presentation and clinical course of patients with Ebstein are quite variable and dependent upon the severity of the malformation. On one end of the spectrum, fetal death can occur. On the other end of the spectrum, patients with mild forms of Ebstein anomaly can have a normal life span without medical or surgical intervention.
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