Ebstein's anomaly is a rare congenital cardiac disease initially described by Wilhelm Ebstein in 1866
In Ebstein's anomaly the tricuspid valve is downwardly displaced into the right ventricle. The posterior and septal leaflets are plastered down into the right ventricular wall. Above the attachment of the valve the ventricle becomes dilated and thin walled - atrialized. Often there is also an atrial septal defect.
Ebstein's anomaly occurs approximately once in 20,000 live births (1) and only 5% of patients survive beyond the fifth decade (1,2).
Echocardiography is the method of choice to diagnose Ebstein's anomaly on its own or in association with other heart defects.
Complications such as right ventricular failure, infective endocarditis, and paradoxical embolism can occur.
Ebstein's anomaly diagnosed in adult life is a benign and stable disease, particularly if the patient is asymptomatic (3). These cases may represent a familial form of Ebstein's anomaly (3).
Last reviewed 01/2018