Ebstein's anomaly is a rare heart defect that's present at birth (congenital). In Ebstein's anomaly, tricuspid valve i.e valve between the chambers on the right side of your heart doesn't work properly. Blood leaks back through the valve, making your heart work less efficiently. Ebstein's anomaly may also lead to enlargement of the heart or heart failure. 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 the following:
• Fatigue and dyspnea
• Palpitations and sudden cardiac death
• Symptoms of right heart failure, such as edema and ascites Physical findings in patients with Ebstein anomaly span a spectrum from subtle to dramatic.
They may include the following:
• Cyanosis and clubbing
• Precordial asymmetry
• Jugular venous pulse
• Arterial pulses: Usually normal but are diminished late in the disease course
• Heart sounds Treatment of Ebstein anomaly is complex and dictated mainly by the severity of the disease itself and the effect of accompanying congenital structural and electrical abnormalities.
Treatment options include medical therapy, radiofrequency ablation, and surgical therapy.
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. According to older observational data, appro Ebstein anomaly is more common in children of white females.Approximately 5% of these patients survive beyond age 50 years. The oldest recorded patient lived to age 85 years.