Ebstein anomaly is a congenital heart defect characterized by downward displacement of the tricuspid valve into the right ventricle. This causes portions of the right ventricle to function as an extension of the right atrium, resulting in right ventricular hypoplasia. Echocardiography can diagnose Ebstein anomaly by measuring the displacement index of the tricuspid valve leaflets and degree of displacement from the atrioventricular ring. The anomaly causes tricuspid regurgitation due to tethering and deformation of the valve leaflets. Severity is classified depending on extent of displacement and right ventricular dysfunction.
3. Normal tricuspid valve
• Normally: Tricuspid valve inserts in the Av
junction : slightly below the insertion of mitral
valve
4. • There is exagerated downward displacement
of the septal and posterior leaflets of the
tricuspid valve into the RV cavity .
• so that…..> a portion of the RV is
incorporated into the RA (i.e., atrialized RV)-
functional hypoplasia of the RV .
5. • Displacement of the annular attachments
(hinges) of the septal and inferior leaflets,
away from the atrioventricular junction.
• It is due to failure of (delamination )
6. • This failure of delamination results in
the leaflets remaining variably adherent to the
underlying right ventricular and septal
myocardium
7.
8.
9.
10.
11.
12.
13. • the septal and inferior leaflets are most
involved.
The anterior leaflet is normal in position.
(displaced in rare cases)
14. • it is important to distinguish pathologic
displacement of the septal leaflet from the
typical valvar off-setting found in the normal
heart.
18. Echo evaluation
4 CH
• The distance between the valvar hinge points
can easily be measured in systole .
• This distance, when divided by the body
surface area (BSA) , is known
as the displacement index.
• index value > 8 mm/m2 reliably diagnose
Ebstein’s malformation .
19. Degree of displacement
• dividing the apical displacement of the septal
leaflet (from the atrioventricular ring) by the
septal length in the apical four-chamber view
(in diastole)
• Mild : ratio < 0.45
• Moderate: 0.5 and 0.6
• Severe : >0.6
32. the vena contracta.
adult
a vena contracta width of less than 3 mm is
associated with mild regurgitation.
• Vena contracta of 8 to 10 mm represents
severe transvalvar regurgitation
33. • Vena contracta diameters that are less
than 10% of the normal annulus considered
mild.
• Vena contracta measuring more than 25% to
30% of the normal annular dimension would
be classified as severe
34. Apical 4ch
• The degree of right atrial and ventricular
enlargement and functional state of the right
ventricular myocardium should be specifically
defined.
37. Other findings……
• patent foramen ovale
• secundum atrial septal defect,IAS aneurysm.
• pulmonary stenosis or atresia.
• Do not forget…Assessment of RV function .