Designed & Managed by Dr. Joseph Vettukattil, Consultant Congenital Cardiologist, Southampton University Hospital, UK
Abnormalities of the tricuspid Valve
2. Ebstein's Anomaly of the Tricuspid Valve
The patho-morphology of Ebstein's malformation include the following
Displacement
of the coaptation point of the right AV valve
Rotational anomaly of the valve leaflets
along the axis of the aorta
Failure of delamination
of individual leaflets
Valve dysplasia
Abnormalities of tension (subcordal) apparatus
Myocardial abnormalities
Abnormal Function of the right ventricle
Variable abnormalities in the Anatomy and function of the systemic ventricle
Co-existent cardiac anomalies (ASD, VSD, PS)
Cartoon demonstrating the displacement of the right Atrioventricular valve towards the apex of the right ventricle in Ebstein's anomaly. Normal atrioventricular junction is defined by the dotted line. To the right is the 3D reconstruction of the cartoon showing the locus of formation of the tricuspid valve.
3D echocardiographic correlate of valfusion resulting in bifoliate closure of tricuspid valve:
(Specimens from prof Anderson)
Figure below shows fusion between the septal and mural leaflet causing bicuspid closure line.
Severe dysplasia of the subcordal apparutus leading to multipple hyphanated attachments to multipple points on the ventricular wall as shown below.
Echocardiographic profile of a normal tricuspid valve viewed from the right atria
2D TOE showing two points of regurgitation from a right AV valve in a patient with Ebsteins.
This video profiles the abnormality of the tricuspid valve very precisely. The valve has two points of coaptation failures: 1. between the septal and mural leaflets. It almost appears to have an extra leaflet formation at the posterior aspect. 2. There is another point of coaptation failure between the anterio-superior (ASL) and the mural leaflets. Use the pause button to stop and review the abnormality more clearly.
Demonstration of bifoliate closure of an Ebstein's valve. Displacement of the leaflets and the atrialization of the RV is clear from this longaxis obleque view from the right atria.
Bifoliate clossure line of a tricuspid valve. Here too there are two points of coaptation failure. Note that the septal leaflet is very dysplastic and immobile. This is compensated by the highly mobile antero-superior leaf let. This leaflet has a tethering which leads to a point of coaptation failure between it and the mural leaflet. The mural leaflet itself is well formed but less mobile.
MPR of a dysplastic tricuspid valve demonstrating the delamination anomaly of both the septal and anterio-superior leaflets.
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Abnormal shelf formation of the ASL in apatient with Ebstein's showing a fixed coaptation failure through which regurgitation ocurs
Partially removed ASL and both atria and ventricle to just demonstrate the point of coaptation in a patient with Ebstein's where there is both stenosis (abnormal sub-cordal apparatus attachements) and regurgitation (coaptation failure)
