topic presentation Ebstein anomaly

1. Ebstein’s Anomaly
T.Ruangpratyakul, MD

2. “Concerning a very rare case of insufficiency of
tricuspid valve caused by a congenital malformation”
Dr. Wilhelm Ebstein, 1866

3. Pathologic Anatomy
• Failure of delamination of leaflets of TV
• Apical displacement of functional TV annulus(SL>PL>AL)
• Dilation of the atrialized portion of the right ventricular
• Anterior leaflet fenestrations, redundancy or tethering
• Dilation of true anatomic TV annulus

4. Failure of delamination

5. fenestration in TV
rudimentary septal leaflets of TV
with it chordae

6. Numerous of muscular stump

7. Associated cardiac defects
• ASD 80-94%
• VSD
• RV outflow tract
obstruction
• PDA
• Coarctation of aorta
• Accessory conduction
pathways >> WPW
syndrome 15-20%
• Left-sided lesions
(uncommon)
• cc-TGA

8. Carpentier Classification
Type A: the volume of the true
RV is adequate
Type B: a large atrialized
component of the RV exists,
but the anterior leaflet of the TV
moves freely
Type C: severe restriction of the
anterior leaflet movement that
can cause significant RV
outflow tract obstruction
Type D: near complete
atrialization of the ventricle

9. Genetic factor
• familial RARE
• NYX 2.5 mutation, 10p13-p14 deletion, 1p34.3-p36.11
deletion
• mutation in gene MYH7

10. Clinical presentation
• Fetuses: abnormal routine prenatal scan (86%)
• Neonates: cyanosis (74%)
• Infants: heart failure (43%)
• Children: incidental murmur (63%)
• Adolescents and adults: arrhythmia (42%), decreased
exercise tolerance, fatigue, or right-sided heart failure

11. Symptoms
• Cyanosis
• Exertional dyspnea
• Fatigue
• Palpitation
• Heart failure
• Paradoxical embolism

12. Physical examination
• Cyanosis
• Widely split 1st and 2nd sound
• Prominent S3 or a loud S4 (S3, S4 gallop)
• Systolic murmur
• Hepatomegaly
• Prominent “a” wave in the distended jugular veins

13. Diagnostic
Modality

14. Chest X-ray
Typical Configuration: globular-shaped heart with a narrow waist

15. Electrocardiography
• Right bundle branch block
• WPW preexcitation
• Supraventricular tachycardia
• Atrial flutter or fibrilation
• Arrhythmogenic atrialized RV
• Deep Q wave in leads V1-4 and inferior leads
• First degree heart block (42%)

17. Echocardiography
• ** Diagnostic test of choice **
• **Apical displacement of septal leaflets at least 8 mm/m2
• Leaflets tethering cause restricted motion
• Marker enlargement of RA and atrialized RA

18. Example of an echocardiogram (4-chamber view, apex
down) of a patient with severe Ebstein’s anomaly showing a
grossly displaced septal leaflet (arrow). The anterior leaflet is
severely tethered and nearly immobile. The functional right
ventricle (RV) is small. ARV indicates atrialized right ventricle;
LA, left atrium; LV, left ventricle; and RA, right atrium.

19. GOSE score
Grade 3-4Poorprognosis!!

20. MRI
• measure RA RV size
and systolic function
• analysis severity of
disease
size &
function of
RV

21. Natural history
• Prenatal: poor prognosis severity relate with RV LV function
• late diagnosis is associated with reduced survival
• adult: arrhythmias is most common presentation(51%)
• predictor: 1.cardio-thoracic ratio >0.65
2.severity of TV displacement
3.NYHA class III or IV
4.cyanosis
5.severe TR
6.younger age at diagnosis

22. Management

23. Neonates and Infants
Indication
of sx
Heart Failure
Cyanosis
Method
Biventricular repair
(Knott-Craig Approach
Right ventricular exclusion
(Starnes Approach)
Total ventricular exclusion
(Modified Starnes Approach)
Heart Transplant

24. Biventricular repair

25. Right ventricular exclusion
1. fenestrated patch closure TV
annulus
2. enlarged ASD
3. placeda systemic-to-
pulmonary shunt

26. Total ventricular exclusion
• free wall of the RV is
resected and closed
primarily or with a
polytetrafluoroethylene
patch.

27. Children and Adult
1.Symptomatic
2.cyanosis
3.paradoxcal embolism
4.decrease exercise
5.increase heart size
6. progress RV dilatation
7.reduced RV function
8.A,V arrhythmia
1.closure of any atrial septal communication
2.correction of associated anomaly
3.performed indicated antiarrhythmia procedures
4.internal plication of atrailized portion of RV
5.repair of TV
6.right reduction atrioplasty
Indication
for surgery
Operative
management

28. Cone Procedure
• Pos
Pre-op Post-op

29. Cone Procedure
A
detached posterior leaflet from annulus
discont. sub-valve apparatus
to free leaflet completely
from it’s displacement into ventricle
A B

30. Cone Procedure
disconnected septal leaflet from annulus
reunited posterior and
septal leaflets with interrupt stitchsC
D

31. Cone Procedure
plication of atrialized RV to recreated
new upward located annuls
posterior annulus plication
for annular reduction
E
F

32. Cone Procedure
new TV reattached to the new annulus
Final shape new tricuspid valve
and closed ASD
G H

33. Adjuncts to Cone repair
anterior leaflet augmentation
with Cor Matrix membrane
triangular patch multiple vertical fenestration

34. Tricuspid valve replacement
• option for who cannot repair
• bioprosthetic valve (not have
thromoembolic complication)
• avoid injury to AV node, RCA
- muscularization of ant. leaflet
not to debulking or resection
- absent septal leaflet
- older >60 yr
- massive RV
- annular dilatation

35. The 1.5-ventricular repair
• Bidirectional cavopulmonary
shunt
• RV severely dilated and poorly
function (EF 35-40%), TV
moderate stenosis (MG > 6
mmHg)
• not for LVED pressure <12,
tranpul. gradient <10,
mean PAP <18
• Disadvantage: pulsation of H&N
vein, facial swelling, AVF in
pulmonary

36. Surgical treatment of
Arrhythmia
• Most common :
atrial fibrillation and
flutter
• prefer biatrial maze
procedure : chronic
AF
• add “cavotricuspid
isthmus” in atrial
flutter

37. Postoperative Care
• epinephrine and milirinone
• minimize RV dilatation, prefer HR 100-120 bpm
• low dose vasopressin helpful with right-side HF
• volume admistration keep RA pressure 10-12 mmHg
• can mild metabolic acidosis as long as UO satisfactory
• NO helpful offset pulmonary vasocontric from inotropic
• D/C med: beta blocker, ACEI, sildenafil (6-8wk), amiodarone(2-3
mo. in arrhythmia pt.)

38. Outcomes

40. • Early mortality 1%
• Survival at 5,10,15,20 yr: 94%, 90%, 86%, 76%
• Survival free for late operation at at 5,10,15,20 yr : 86%,
74%, 62%, 46%
• 83% NYHA class I or II
• 34% no cardiac medication
• problem: late reoperation, rehospitalization and atrial
tacchyarrhythmias

41. THANK YOU