ebstein's anomali

1. EBSTEIN’S ANOMALI
Dr.Tanvir Rahman
MS(CTS) final part student
NHFH & RI

2. Ebstein’s anomaly is a congenital malformation
of the heart that is characterized by
• Delamination failure of TV
leaflets(adherence of tricuspid leaflet to
underlying myocardium)
• Apical displacement of functional
annulus(septal>anterior>posterior)
• Atrialization & Dilatation of atrialized
portion of RV
• Redundency fenestration and tethering of
anterior leaflet
• Dilatation of true tricuspid annulus
• Variable ventricular myocardial
dysfunction

3. History and background
• Wilhelm Ebstein first described a patient with
cardiac defects typical of Ebstein anomaly in
1866.
• In 1927, Alfred Arnstein suggested the name
Ebstein's anomaly for these defects.
• It presented an ongoing challenge since its
initial repair attempts in 1958
• First successful replacement in 1963 by
Barnard and Schrire

4. epidemiology
The natural course of the disease varies according
to the severity of tricuspid valve displacement.
Patients presenting in infancy generally have severe
disease and unfavorable prognosis.
Mean age of presentation is in the middle teenage
years.
approximately 5% of these patients survive beyond
age 50 years.
The oldest recorded patient lived to age 85 years.

5. pathophysiology
The ultimate hemodynamic consequences of Ebstein’s
anomaly is heart failure due to
•malformed tricuspid leaflets leading to regurgitation(The
severity of regurgitation depends on the extent of leaflet
displacement)
•The atrialized portion of the right ventricle(although
anatomically part of the right atrium) contracts paradoxically
Leads to stagnation of blood in RA during RV relaxation and
causes a backward flow of blood into the right atrium during
RV systole
And deformed TV leaflet may lead to RVOT obstruction and
cyanosis

6. presentation
• Patients can have a variety of symptoms related
to the anatomical abnormalities of Ebstein’s
anomaly and their hemodynamic effects or
associated structural and conduction system
disease.
SOB on exertion
Occasional palpitation
fatigue
Features of heart failure

7. Physical examination findings
• Cyanosis
• JVP- normal or large V wave
• Liver palpable but not pulsatile
• Ascitis & Peripheral oedema at advanced stage
• Apex beat shifted to left
• Left parasternal heave
• Wide splitting of both 1st
and 2nd
heart sound
• Soft Systolic murmur over left parasternal area due to TR

8. investigations

9. ECG
ECG-
P pulmonale,
RBBB,
SVT, paroxysmal SVT,
atrial flutter,
atrial fibrillation,
ventricular tachycardia

10. Chest Xray
• Globular shaped heart (due
to RA hypertrophy and
outward and upward
displacement of RVOT)
• Oligemic lung field

11. ECHO
Echocardiogram(2D/3D)
TV anatomy
(annulus,leaflets,leaflet
attachments,coaptation,jet flow)
RVOT
PV anatomy
ASD/VSD size and flow
direction
all chamber anatomy and size
measurement
RV and LV function

12. Cardiac cath (haemodynamic cath)
done in selective cases
particularly in LV dysfunction,
and when
BDCP shunt is planned
to see LV and RV pressure

13. Other investigations
CT angiogram
MRI-quantitive
measurement of LV and
RV size
Intra operative TEE
Invasive ECG

14. classification
• Based on morphology
of RV and TV
(Carpentier’s
classification-1988)

15. Risk Assessment Great Ormond Street
Echo Score
Area of (RA + aRV)
Area of (RV + LV + LA)
1 <0.5 8%
2 0.5-1.0 8%
3 (acyanotic) 1.1-1.4 10% E, 45% L
3 (cyanotic) 1.1-1.4 100%
4 >1.5 100%
GOSE Ratio Mortality
Score

16. Treatment options
• Mostly Surgical management
biventricular repair approach
Univentricular /RV exclusion approach
Heart lung transplant
Medical treatment can be given for symptom
alleviation and control of heart failure

17. Indication of surgical management
• Severe symptomatic
• NYHA Class III/IV
• Severe cyanotic
• Paradoxical embolism
• Cardiomegaly
• Systolic dysfunction

18. Relative contra indication of surgery
• Relative Contra indications:
• Older age(>50 years)
• Moderate pulmonary hypertension
• LVEF <30%
• Complete failure /poor delamination of leaflets
• Severe RV enlargement
• Severe dilatation of true tricuspid annulus

19. Surgical Procedures
• Danielson (valvuloplasty by annuloplasty with/without
annuloplasty ring +horizontal plication of non
functional/atrialized portion of RV)
• Modified Danielson( annular remodeling)
• Carpentier ( rotation valvuloplasty –annuloplasty)
• Cone procedure
• Bichell procedure
• TVR (without plication)
• Starnes procedure (single ventricle palliation strategy)

20. Cone procedure of TV repair -the latest option
• Surgical Delamination Of Fibrous &
Muscular Attachments
• Clockwise rotation of the leaflets and
Suturing margin of PL to SL to form a
cone
• Vertical Plication of Large Atrialized
RV
• Annular Reduction and Re suturing of
leaflets
• Complete reconstruction with partial
closure of ASD/PFO

21. Clockwise rotation of the leaflets and Suturing margin of PL to SL to
form a cone

22. Plication of Large Atrialized RV
• Decreases tension at annulus
• Beware of coronary arteries!!

23. Annular
reduction
Annular Reduction and Re suturing of leaflets

24. Advantage of Cone repair
Leaflet to leaflet coaptation
Re constractedTV reattached to true annulus
Hinge part of valve is in normal anatomical position
Plication of thin transparent atriaalized RV eliminates chance
of dyskinesia
Excision of redundant RA
Vertical plication allow mentainance of near normal
ventricular anatomy

25. Heart transplantation
Indications:
•Severe biventricular dysfunction(RV dilatation
and dysfunction with severe LV dysfunction
LVEF <25%)
•Left ventricular dilatation and dysfunction with
MR

26. THANK YOU

28. Univentricular/RV exclusion approach (Starnes and Colleagues)
• Patch closure of TV (4-5 mm
fenestrated patch for RV
decompression as it progressively fills
with thebesian venous return)
• Enlargement of interatrial connection
• Placement of systemic to pulmonary
arterial shunt
• RA reduction
• Ligation of MPA (if there is
incompetent PV with patent RVOT

30. Modified Starnes/total RV exclusion(Sano and Associates)
Resection of Free wall of RV followed by
primary closure
PTFE closure