Ebstein's anomaly echocardiogram
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This document discusses the echocardiographic features used to evaluate Ebstein's anomaly of the tricuspid valve. It describes how to assess the displacement and morphology of the tricuspid valve leaflets, degree of tethering, and dilation of the cardiac chambers. Cut-off values are provided to define abnormalities. The document also reviews how to evaluate tricuspid regurgitation and the anatomy of the tricuspid valve annulus, chordae, and right ventricle outflow tract. Assessment of left ventricular function is also mentioned. Evaluation of Ebstein's anomaly by 2D, M-Mode, Doppler and 3D echocardiography is covered. Scoring systems for evaluating severity and prognosis are
Ebstein's anomaly echocardiogram
- 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
- 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.
- 10. Tethering
- 11. Chamber enlargement Gose score
- 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%
- 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.
- 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
- 30. A4C view
- 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
- 37. 3d ECHO
- 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
- 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)
- 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
- 47. SAS SCORE --- 0 TO 10 > 5 --- NO SURVIVORS < 3 --- 91% SURVIVAL
Editor's Notes
- #14: 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
- #32: 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).
- #33: 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
- #46: 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.