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Ebstein’s anomaly is a congenital heart defect in which the opening of the tricuspid valve is displaced towards the apex of the right ventricle of the heart.
The annulus of the valve is still in the normal position. The valve leaflets, however, are to a varying degree, attached to the walls and septum of the right ventricle. There is subsequent ‘atrialization’ of a portion of the morphologic right ventricle (which is then contiguous with the right atrium). This causes the right atrium to be large and the anatomic right ventricle to be small in size.
There may be an increased risk of this abnormality in infants of women taking lithium during the first trimester of pregnancy, and in those with Wolff-Parkinson-White syndrome.
Ebstein’s anomaly was named after Wilhelm Ebstein.[1][2]
While Ebstein’s anomaly is defined as the congenital displacement of the tricuspid valve towards the apex of the right ventricle, it is often associated with other abnormalities.
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