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 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.
 Tricuspid valve leaflet displacement
septal leaflet
 Normally --- apical displacement +
 Ebstein’s --- exaggerated displacement
 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
 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
 Apical displacement of the septal leaflet from the
insertion of the anterior leaflet of the mitral valve by at
least 8 mm/m2
Morphology of leaflets
 Septal & posterior -
 Displacement
 Dysplasia
 Absence
 Anterior
 Elongation, redundancy , sail like appearance
 Distal attachments
 Mobility
 Fenestrations
Tethering of the tricuspid valve
 at least 3 accessory attachments of the leaflet to the
ventricular wall, causing restricted motion of the
leaflet.
Tethering
 Chamber enlargement
 Gose score
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.
GOSE score
Grade Ratio Mortality
1 <0.5 0%
2 0.5-0.99 8%
3
(acyanotic)
1-1.49 10% (neonatal)
45% (later)
3
(cyanotic)
1-1.49 100%
4 >1.5 100%
TR ASSESMENT
 spectral & colour
 direction, no, origin (fenestrations)
Tricuspid anulus
 Size – z score
comparison with mitral v annulus
 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.
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.
RVOT
 RVOT OBS
 Aneurysmal dilation - equal to or greater than twice
the aortic root diameter (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
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.
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
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
PSAX
A4C view
 Displacement index
 septal & ant leaflet
 Gose score
 BV function
A4C view
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.
CARPENTIER classification
dd
 Tricuspid dysplasia – nodular thicking and rolling
edges of leaflet with out displacement
 Ungaurded tricuspid valve – all 3 leaflets are absent
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
3d ECHO
Pre op assesment
 Prognosis
 Tv repair vs replacement
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
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)
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
SAS SCORE --- 0 TO 10
> 5 --- NO SURVIVORS
< 3 --- 91% SURVIVAL

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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.
  • 3.  Tricuspid valve leaflet displacement septal leaflet  Normally --- apical displacement +  Ebstein’s --- exaggerated displacement
  • 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
  • 8. Morphology of leaflets  Septal & posterior -  Displacement  Dysplasia  Absence  Anterior  Elongation, redundancy , sail like appearance  Distal attachments  Mobility  Fenestrations
  • 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.
  • 13. GOSE score Grade Ratio Mortality 1 <0.5 0% 2 0.5-0.99 8% 3 (acyanotic) 1-1.49 10% (neonatal) 45% (later) 3 (cyanotic) 1-1.49 100% 4 >1.5 100%
  • 14.
  • 15. TR ASSESMENT  spectral & colour  direction, no, origin (fenestrations)
  • 16. Tricuspid anulus  Size – z score comparison with mitral v annulus
  • 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
  • 28. PSAX
  • 29. A4C view  Displacement index  septal & ant leaflet  Gose score  BV function
  • 31.
  • 32.
  • 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
  • 38.
  • 39. Pre op assesment  Prognosis  Tv repair vs replacement
  • 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

  1. 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
  2. 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).
  3. 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
  4. 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.