synopsis in echocardiographic Evaluation of Ebstein anomaly

1. Ebstein Anomaly
BY
Dr.Aliaa Shaban
Lecturer of cardiovascular Medicine
Tanta University
Egypt

2. • primary disorder of the tricuspid valve.

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

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.

15. Classification of severity

16. • Type A , B= Mild form
• Type C ,D =severe form

17. 4ch, RV inflow, PSAX

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

27. Morphology of the tricuspid valve
• Tethering
• regurgitation

29. Tethering

30. Parasternal short axis
Displaced septal
leaflet

31. Tricuspid incompetence

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.

35. Parasternal short axis

36. PLAX

37. Other findings……
• patent foramen ovale
• secundum atrial septal defect,IAS aneurysm.
• pulmonary stenosis or atresia.
• Do not forget…Assessment of RV function .

39. Grading in neonates