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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 anomaly is a rare heart defect in which parts of the tricuspid valve are abnormal. The tricuspid valve separates the right lower heart chamber (right ventricle) from the right upper heart chamber (right atrium). In Ebstein anomaly, the positioning of the tricuspid valve and how it functions to separate the 2 chambers is abnormal. The condition is congenital, which means it is present at birth. Ebstein anomaly is an abnormality in the tricuspid valve. The tricuspid valve separates the right atrium (the chamber that receives blood from the body) from the right ventricle (the chamber that pumps blood to the lungs).
Mild forms of Ebstein's anomaly may not cause symptoms until later in adulthood. If signs and symptoms are present, they may include:
• Shortness of breath, especially with exertion
• Fatigue
• Heart palpitations or abnormal heart rhythms (arrhythmias)
• A bluish discoloration of the lips and skin caused by low oxygen (cyanosis)
True prevalence is unknown because mild forms frequently are undiagnosed. Currently, with wide application of echocardiography, more cases are being diagnosed. Ebstein anomaly probably accounts for 0.5% of cases of congenital heart diseases.
