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Ebstein's anomaly echocardiogram
1.
2. Tricuspid valve leaflet displacement
Morphology & Tethering of the TV leaflets
Tricuspid valve motion and coaptation
Dilatation of chambers
TR assesment
Dilation of the right atrioventricular junction (true
tricuspid annulus)
Dilation of right ventricular outflow tract
Exaggerated tricuspid anulus motion
Left ventricular function and dimension.
4. Ebsteins – 7 to 50mm,
Normal tricuspid valve - 0 to 10 mm,
Secundum atrial defect - 2 to 14 mm
Severe TR- 2 to 15 mm
overlap with values for Ebstein ' s anomaly.
So, cut off is
15mm--- < 14yr
20mm--- >14 yr
5. indexed to body surface area
Ebstein 's anomaly --- 8.5 to 11.4 ,median 8.4mm/m2
Normal --- 0 to 6.3, median 3.0mm/m2;
ASD-- 0.9to 7.5, median 5.0mm/m2;
TR --- 1.1 to 7.5, median 4.0mm/m2
CUT OFF IS --- 8 MM/M2
6.
7. Apical displacement of the septal leaflet from the
insertion of the anterior leaflet of the mitral valve by at
least 8 mm/m2
9. Tethering of the tricuspid valve
at least 3 accessory attachments of the leaflet to the
ventricular wall, causing restricted motion of the
leaflet.
12. Ebstein’s: Echocardiographic
Severity index
Celermajer et al
grade (1 to 4)
Gose score(Great
Ormond Street Score for
neonates)
Celermajer et al. Outcome in neonates with
Ebstein’s anomaly. JACC 1992; 19:1047-8.
17. Occasionally = tissue bridge forms connecting leading
edges of septal and anterior lt turning the commissure
into a key hole --- TS
Even imperforate TV if closes completely.
18. Sub valvular apparatus
Short chordae may attach septal leaflet to ventricular
septum
Sometimes chordae may absent with insertion of
leaflet directly to the ventricular septum.
19. RVOT
RVOT OBS
Aneurysmal dilation - equal to or greater than twice
the aortic root diameter (20%).
20. interatrial communication - 80% to 94%
Pda
Bicuspid or atretic aortic valves.
Pulmonary atresia or hypoplastic pulmonary
artery, PS
Subaortic stenosis, COA
MVP , accessory mitral valve tissue
Muscle bands of LV ,double orifice MV.
VSD
LV non compaction
21. M mode
Delayed closure of the tricuspid valve compared with
that of the mitral valve.
delay in EA -- > 50 msec
N --- 20-30 msec
Paradoxical motion -IVS
Increased RVdimension
Increased excursion of AML of the mitral valve
greater the delay in tricuspid valve closure, the more
severe the disease.
22.
23.
24.
25.
26. PLAX
RV vol overload
Paradoxical septal motion
Free edges of TV seen in RV
Displaced origins of leaflets
Distinguish between anatomic & functional annulus
Chordal attachments of ATL
27. PSAX
Septal & ant leaflets seen, adherent to septal surface
Length of ATL & its mobility
Excessive size of ATL-systolic obstruction of RVOT
Functional & anatomical pul atresia
In neonates with severe ebsteins – pulmonary annulus
& branches are small
33. SUBCOSTAL VIEWS
Coronal view- degree of adherence of septal leaflet to
ven myocardium & degree of elongation of ATL
Superior angulation- RVOT – degree of encroachment
of ATL on RVOT.
Sagittal view- sail like ATL & abnormal PTL
ENFACE view - from coronal view, 30 - 45 degrees
clock wise rotation- all leaflets are seen.
35. dd
Tricuspid dysplasia – nodular thicking and rolling
edges of leaflet with out displacement
Ungaurded tricuspid valve – all 3 leaflets are absent
36. 3D ECHO
Enables reconstruction of TV
Visualise all 3 leaflets at same time – enface
view(surgical view)
Better understanding of anotamy
Degree of delamination
Sub valvular apparatus
40. Successful Monocusp Repair
Freely mobile ATL
Body of Leaflet and the Leading Edge can reach the
septum
No Direct papillary muscle insertions
Single Central Jet of TR
No TV Chordal attachments in the RVOT
Adequate Postop Functional RV size
41.
42. Unfavorable Features for Monocusp Repair
Tethered ATL with restricted mobility of Body of
Leaflet and the Leading Edge
Direct papillary muscle insertions onto valve tissue
(no chordae)
Multiple Jets of TR (fenestrations)
TV Chordal attachments in the RVOT (near the PV)
43.
44. Post op assesment
Early post op –
pericardial effusion, mediastinal hematoma,
Intracardiac thrombus
BV function, RWMA
gr across TV,
TR assesment( residual)
Long term –
RA, RV enlargement,
TV fuction
45.
46.
47. SAS SCORE --- 0 TO 10
> 5 --- NO SURVIVORS
< 3 --- 91% SURVIVAL
Editor's Notes
Involves calculating the ratio of the combined area of the RA and atrialized RV to that of the functional RV and L heart in a 4 chamber view at end diastole
Even grade 3 carries with it a separate, late risk of death even in the acyanotic neonate
Apical 4-chamber, 2-dimensional echocardiogram in a patient with Ebstein anomaly shows displacement of the tricuspid valve toward the apex of the right ventricle (RV) and tethering of the septal leaflet to the interventricular septum (arrow).
Apical 4-chamber image from 2-dimensional (2D) echocardiography (Echo) in a patient with severe Ebstein anomaly shows displacement of the tricuspid valve towards the apex of the right ventricle (RV) more extreme than that shown in the previous 2 images. The atrialized part of the RV is more dilated and the tethering of the septal leaflet extends further toward the apex
these apical four-chamber images show two patients
with ebstein’s malformation. the case illustrated in the upper panels
shows a valve that is freely mobile (upper left panel) and colour flow
mapping (upper right panel) revealed that there was only a single,
central jet of regurgitation. this patient subsequently had a successful
valve repair with only mild residual tricuspid regurgitation and no
stenosis. the case shown in the lower panels displays a large muscular
insertion to the middle of the anterosuperior leaflet (lower left) and
multiple fenestrations and sites of regurgitation. the tethering and
multiple origins of regurgitant flow dramatically decrease the chance
for successful repair.