Echo in Ebstein's anomaly of the tricuspid valve

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Editors: Eli V. Gelfand, MD and Keri Shafer, MD (Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA)

Ebstein's Anomaly of the Tricuspid Valve

Ebstein's Anomaly is the apical displacement of the tricuspid valve into the right ventricle. For more see: Ebstein's Anomaly of the Tricuspid Valve

Graphical represntation of Ebstein's Anomaly from the Mayo Clinic website (note there is also an ASD on this diagram)

Echo functions in Ebstein's Anomaly

Echocardiographic evaluation of Ebstein's must include evaluation of:

extent of apical displacement and attachment location of the leaflets
degree of tricuspid regurgitation
morphology of the anterior leaflet
presence or absence of right ventricular outflow tract obstruction
degree of atrialization
surgical options
presence of mitral valve prolapse
associated anomalities including Atrial Septal Defect and less commonly Ventricular Septal Defect

Special Echo techniques in Ebstein's Anomaly

Measurement of the distance between the insertion sites of the tricuspid leaflets
Doppler should be used to determine the degree of tricuspid regurgitation

Ebstein's Anomaly is best seen in the apical four chamber view

Echocardiographic representation from in various views

Apical 4-chamber:

Apical continuous-wave doppler:

Short axis view (notice the tethering of the tricuspid valve):

Below in a video clip is an apical 4-chamber transthoracic view, showing apically-displaced leaflets of the tricuspid valve. Note substantial tricuspid regurgitation, which originates close to the apex, where the abnormal leaflets actually coapt:

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