Ebstein's anomaly is a congenital heart defect in which the septal and posterior leaflets of the tricuspid valve are displaced towards the apex of the right ventricle of the heart. Ebstein's anomaly is a rare disease involving the right side of the heart. This disorder occurs when the tricuspid valve, that divides the two chambers of the right side of the heart, does not form correctly.
If the irregularity is severe at birth, it may require immediate surgery. In adults with milder cases, treatment may include medications or special procedures to manage arrhythmia. People with Ebstein anomaly should be prescribed antibiotics before surgery or dental procedures to protect against endocarditis. If medication no longer controls symptoms or if the heart enlarges and loses efficiency, surgery may be required. The surgery may either repair the tricuspid valve or replace it with an artificial valve. It also may involve repairing any associated condition such as atrial septal defect. If arrhythmia is present, radiofrequency ablation, a procedure in which a burst of energy destroys an abnormal electrical pathway in the heart, may be necessary. Rarely, a permanent pacemaker will be implanted. Adults with Ebstein anomaly should be followed regularly by a cardiologist with a special interest in adult congenital heart disease. Yearly echocardiograms are necessary in all but the mildest cases.
Hemodynamic consequences of this lesion are directly related to the severity of the leaflet displacement and the resultant tricuspid valve regurgitation. In the case of mild displacement and mild valvar regurgitation, the patient may be asymptomatic for many years. If, however, the leaflet displacement and valvar regurgitation are severe, pulmonary blood flow is decreased, the right atrium becomes dilated, blood is shunted right to left across an ASD or PFO, and the patient may become cyanotic. Congestive heart failure may also develop secondary to a small functional right ventricle and decreased right ventricular compliance. Additional problems in these patients include an association with paroxysmal supraventricular tachycardia (SVT), which occurs in 25-50% of patients, and 5-10% of these patients have Wolff-Parkinson-White (WPW) syndrome