In Ebstein's anomaly, the septal and posterior leaflets of the tricuspid valve are downwardly displaced into the right ventricular cavity and the normally situated anterior leaflet may have abnormal leaflet attachments. As a result, the right side of the heart is abnormally partitioned into a right atrium, an atrialized portion of the right ventricle, and a right ventricular cavity. A communication between the left and right atrium is often present. These abnormalities result in abnormal right ventricle function and a leak in the tricuspid valve. In addition, 20% of patients with Ebstein's anomaly have accessory electrical tissue which can cause episodes of heart racing, a condition known as Wolff-Parkinson-White syndrome (WPW).
The clinical manifestations of Ebstein's anomaly are quite variable. Patients with the most severe form of this disease may present with severe congestive heart failure and cyanosis at birth while mild cases may not be discovered until adulthood.
The management of Ebstein's anomaly depends on symptoms, the age of the patient, and the anatomy. Surgery is indicated when there is congestive heart failure from a leak in the tricuspid valve, or significant cyanosis. There is less certainty about the need for surgery in a patient with no symptoms. Some patients with Ebstein's anomaly are free of symptoms until the development of an arrhythmias. These patients may be candidates for both surgical repair of Ebstein's and specific treatment of the arrhythmia. Patients with supraventricular tachycardia from WPW syndrome may be treated with RF ablation; however, this procedure is usually more difficult in Ebstein's anomaly because of the abnormal location of the tricuspid valve.
There are several different surgical options for Ebstein's anomaly. Tricuspid valve reconstruction is preferred, if feasible, but requires the expertise of a surgeon who has established experience with this operation. At our institution, the operation consists of tricuspid valve reconstruction with plication of the atrialized portion of the right ventricle and, if present, closure of the communication between the right and left atrium. The anterior leaflet of the tricuspid valve is incised and repositioned along the newly established tricuspid valve annulus to produce a monocusp tricuspid valve. Tricuspid valve replacement is an alternative when repair is not feasible.
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