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Ebstein’s anomaly is a rare congenital malformation of the right ventricle and tricuspid valve which is characterized by several features that can exhibit an infinite spectrum of malformation.  The abnormalities include: i) adherence of the tricuspid leaflets to the underlying myocardium (failure of delamination); ii) anterior and apical rotational displacement of the functional tricuspid annulus (septal &gt; posterior &gt; anterior leaflet); iii) dilatation of the right atrioventricular junction (the true tricuspid annulus) with variable degrees of hypertrophy and thinning of the wall; iv) dilatation of the “atrialized” portion of the right ventricle; and v) redundancy, fenestration, and tethering of the anterior leaflet.

journal of cardiac critical care tss

Successful One and One-half Ventricular Circulation with Bioprosthetic Tricuspid Valve Replacement and Total Chordal Preservation in a Patient with Ebstein’s Anomaly: A Video Presentation