Ebstein anomaly is a congenital malformation of the heart that is characterized by apical displacement of the septal and posterior tricuspid valve leaflets, leading to atrialization of the right ventricle with a variable degree of malformation and displacement of the anterior leaflet.
Patients can have a variety of symptoms related to the anatomic abnormalities of Ebstein anomaly and their hemodynamic effects or associated structural and conduction system disease, including Cyanosis, fatigue and dyspnea, palpitations and sudden cardiac death , symptoms of right heart failure, such as edema and ascites, brain abscess due to right-to-left shunt, bacterial endocarditis, paradoxical embolism, stroke, and transient ischemic attacks .
This rare heart condition accounts for only 1% of congenital heart defects but its impact is significant, especially to those directly affected. About 10% of cases are associated with chronic maternal lithium use. About 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. The natural course of the disease varies according to the severity of tricuspid valve displacement. Patients presenting in infancy generally have severe disease and unfavorable prognosis. Mean age of presentation is in the middle teenage years.
Treatment of Ebstein's anomaly depends on the severity of the defect and your signs and symptoms. The goal of treatment is to reduce your symptoms and avoid future complications, such as heart failure and arrhythmias.Tricuspid valve repair which involves reducing the size of the valve opening and allowing the existing valve leaflets to come together to work properly. This procedure is usually done when there's enough valve tissue to allow for repair. A newer technique for the repair of Ebstein's anomaly, called the cone procedure, uses the existing leaflets to form a cone that works like a valve. Another procedure that doctors may perform during valve repair or replacement surgery is called a maze procedure. It's used to help correct fast heart rhythms in the atrium.