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1. Ebstein’s Anomaly

2. Introduction
• Ebstein’s anomaly is a
rare congenital heart disorder
occurring in ≈1 per 200 000 live births
<1% of all cases of congenital heart disease.
Equal male: female occurrence

3. • Described by Wilhelm Ebstein in 1866 in a report titled,
“Concerning a very rare case of insufficiency of the tricuspid valve
caused by a congenital malformation.”
• A 19-year-old cyanotic man with dyspnea, palpitations, jugular
venous distension, and cardiomegaly.
• Autopsy-
 An enlarged and fenestrated anterior leaflet of the tricuspid valve.
 Posterior and septal leaflets were hypoplastic, thickened, and
adherent to the right ventricle.
 Thinned and dilated atrialized portion of the right ventricle, an
enlarged right atrium, and a patent foramen ovale.
• By 1950, only 3 cases of this anomaly had been published.

4. Tricuspid Valve Anatomy
• The tricuspid valve is the most apically (or
caudally) placed valve with the largest orifice
among the four valves.
• The tricuspid annulus is oval-shaped and when
dilated becomes more circular.
• 20% larger than MV annulus .
• Normal TV annulus= 3.0 3.5 cm

7. Leaflets
• The tricuspid valve has three distinct leaflets
described as septal, anterior, and posterior.
• The septal and the anterior leaflets are larger.
• The posterior leaflet is smaller and appears to be
of lesser functional significance.

8. • The septal leaflet is in immediate proximity of
the membranous ventricular septum, and its
extension provides a basis for spontaneous
closure of the perimembranous ventricular
septal defect.
• The anterior leaflet is attached to the
anterolateral margin of the annulus.

9. Papillary Muscles & Chordae
• There are three sets of small papillary muscles, each
set being composed of up to three muscles.
• The chordae tendinae arising from each set are
inserted into two adjacent leaflets.
• The anterior set chordae insert into half of the septal
and half of the anterior leaflets.
• The medial and posterior sets are similarly related
to adjacent valve leaflets.

10. • The hallmark definition of Ebstein anomaly is
displacement of the annular attachments (hinge
points) of the septal and inferior (posterior)
leaflets.

11. Embryology
• During normal development
A layer of endomyocardium separates or delaminates,
from the underlying ventricular myocardium, and loses
its muscular character
Delamination begins at the tips of the leaflets and
proceeds toward the AV junction.
A normal, completely delaminated, leaflet will have a
hinge point at or near the anatomic tricuspid valve
annulus

12. Normal Delamination (Separation) of
the TV from the RV Myocardium

13. Embryology- Ebstein’s Anomaly
• The failure of delamination
• Resulting in leaflets that variably adhere to the
underlying RV myocardium
• Characteristic displacement of the attachments of
the valve and rotates its functional orifice away from
normal position and toward the RV apex or into the
RV outflow tract (RVOT) in the most severe cases.
• Anterior and Apical Rotation of the Functional
Orifice.

14. Spectrum of Failed TV Delamination
seen in EbsteinAnomaly

15. • Location of the orifice of the abnormal valve, shown by the green
ovals, as observed in the series of hearts examined.
• The valvar orifice is displaced rotationally, rather than in a
downward fashion.

16. Anterior leaflet
• The anterior leaflet forms at a
different stage of development.
• Its hinge point remains in
normal position at the AV
groove.
• redundant and has been
described as “saillike”
• It can be “muscularized” in the
medial and inferior portion of
the leaflet close to the base of the
heart.

17. Anatomy: Ebstein’s anomaly
• A malformation of the tricuspid valve and right ventricle
characterized by
(1) adherence of the septal and posterior leaflets to the
underlying myocardium (failure of delamination, namely
splitting of the tissue by detachment of the inner layer during
embryologic development)
(2) downward (apical) displacement of the functional annulus
(septal>posterior>anterior)
(3) dilation of the “atrialized” portion of the right ventricle,
with various degrees of hypertrophy and thinning of the wall

18. (4) redundancy, fenestrations, and tethering of
the anterior leaflet
(5) dilation of the right atrioventricular junction
(true tricuspid annulus).

20. Right Ventricle Involvement
• The RV dysfunction is due
abnormal embryonic process of delamination
functional RV volume is smaller than normal
The RV myocardium located
above the orifice becomes thin or
“atrialized” and dysfunctional.
In contrast, the myocardium
below the functional valve
possesses more normal wall
thickness and trabeculae
This region is below the anatomic
AV groove, but proximal to the
functional valve in the region of
the RV with few trabeculae
But the RV dysfunction is
universal

23. Etiology

24. Environmental factors
• More common in twins.
• Family history of congenital heart disease.
• Maternal exposure to benzodiazepines.
• Maternal lithium therapy.
• Most cases are sporadic.

25. Genetic Factors
• Rare cases of cardiac transcription factor
NKX2.5 mutations, 10p13-p14 deletion, and
1p34.3-p36.11 deletion have been described in
the anomaly .
• Postma et al. reported the results of a mutational
analysis in a cohort of 141 unrelated probands
with Ebstein anomaly; eight patients had a
mutation in the MYH7 gene and six also had LV
noncompaction..

28. clinical examination
• Infants with severe Ebstein anomaly have profound cyanosis.
• Cardiac examination usually reveals no lifts or thrills and
normal first and second heart sounds.
• Split first and second sounds and/or third and fourth heart
sounds occur.
• Rarely, in patients with a large, hypermobile anterior leaflet
multiple sounds may occur.
• This “multiplicity of sounds” is unusual (despite previous
descriptions that this is a “classic” finding in patients with
Ebstein anomaly).

29. • Severe tricuspid regurgitation causes a
holosystolic murmur along the left lower sternal
border.
• An ejection murmur of RVOT obstruction can
also be present.

30. • In patients with severe cyanosis, the pulmonic
component may not be detected due to
decreased outflow.
• Because the massively enlarged heart occupies
much of the chest, variable degrees of lung
hypoplasia may be present

31. • Signs and symptoms of Ebstein anomaly later in life are
usually threefold:
(1) murmur,
(2) exertional dyspnea or cyanosis,
(3) palpitations from new-onset arrhythmia
• Cyanosis may be evident but is subtle.
• Jugular venous distension is not common because the large
RA and atrialized RV usually dissipate the “V” wave.
• The V wave can be seen in patients with severe tricuspid
regurgitation without an interatrial shunt. Hepatomegaly is
uncommon for similar reasons.

32. Diagnosis

33. • ECG
• Tall, wide P waves (because of R A enlargement)
• Himalyan P waves (>5mm)

34. • PR interval prolongation-
common
most often due to RA enlargement and long
intra-atrial conduction times.
• PR interval may be short- when conduction is
through accessory pathway.

35. • QRS complex-
May be prolonged
Complete or incomplete RBBB (because of delay
in conduction through specialized conduction
tissue)
May be fragmented (abnormal conduction
through atrialised RV)

36. • Ebstein’s anomaly pts may develop:
• atrial tachycardia, atrial flutter, intra-atrial
reentrant tachycardia, atrial fibrillation, AV
node reentrant tachycardia, and ventricular
arrhythmias

37. • P wave amplitude in lead V1 is > 1.5 mm, suggestive of right atrial
enlargement.
• There is an rSR’ pattern in V1, which is consistent with right ventricular
conduction delay and suggestive of right bundle branch block (RBBB).

38. • Ebstein anomaly with Wolff–Parkinson–White (pre-excitation).
 the right atrial enlargement seen best in lead V2.
 In lead I, there is a short PR interval with slurring of the upstroke of
the QRS (delta wave) consistent with pre-excitation

41. Chest Radiography
• Cardiomegaly (Rounded or Box-like contour )
• Decreased pulmonary vasculature
• The infundibulum either straightens the left cardiac
border or forms a conspicuous convex shoulder.
• The most consistent and dramatic radiologic feature
is the right atrial silhouette, which is almost always
enlarged.

44. Echocardiography
• Two-dimensional echocardiography defines
the anatomic variation of the tricuspid valve
the function of the RV
other potential intracardiac anomalies
• Diagnosis is made by recognition of the failed
delamination of the TV apparatus and rotation
of the valve hinges and orifice toward the RVOT

46. Echo: Apical Displacement Index
• Measured from the
insertion point of the
anterior mitral leaflet to the
hinge point of the tricuspid
septal leaflet (where it
begins to delaminate).
• A displacement index >8
mm/m2 supports a
diagnosis of Ebstein
anomaly

47. • The small white
arrows highlight the
separation between
the septal insertions
of the anterior mitral
and septal tricuspid
leaflets.
• The absolute
distance between the
insertions was 13
mm.
• Patient's body
surface area was 1
m2 . Therefore, the
displacement index
in this case was equal
to 13 mm/m2

48. Celermajer Index
• Celermajer et al. described an echocardiographic grading
score for neonates with Ebstein’s anomaly, the Great Ormond
Street Echocardiography (GOSE) score, with grades 1 to 4.
• This classification is particularly helpful with neonatal
Ebstein’s anomaly for prognosis.

51. Assessment of Tricuspid Valve
Regurgitation in Ebstein Anomaly
• In Ebstein anomaly, the large compliant RA and
atrialized RV absorb the regurgitant volume
systemic venous flow reversals less useful.
hepatomegaly and jugular venous distention are
usually absent
• Due to the anterior and apical displacement of the
functional tricuspid valve orifice, the orientation of
the regurgitant jets may not be completely visualized
in the apical four-chamber view.

52. • In young patients-the subcostal windows
• In older patients-parasternal short axis or anteriorly
angled apical
• If there is a single regurgitant jet the diameter of the
vena contracta and the density of the signal can provide
a convenient and reproducible method for defining
tricuspid regurgitation.
• The density of the tricuspid regurgitation spectral
Doppler signal may also be useful. A very dense signal
usually is associated with severe regurgitation.

54. • Color Doppler to evaluate RVOT obstruction,
and intracardiac shunts.

55. Cardiac MRI
• 1. MRI is preferred for quantitative measurement of
right atrial and RV size and systolic function.
• 2. Provides complimentary information about TV
anatomy .
• 3. Axial imaging provides the most reliable
information about atrialized RV volume.
• 4. The ability to create 3D images may also provide
greater delineation of disease severity.

57. Cardiac Catheterization
• No longer required to make/confirm the
diagnosis
• The most diagnostic characteristic- Typical atrial
pressure & ventricular intracardiac ECG in the
atrialized portion of the RV
• Elevated RAP
• R-L atrial shunting with systemic desaturation
• Elevated RVEDP

58. Carpentier’s classification
• In 1988, Carpentier et al. proposed the following
classification of Ebstein’s anomaly –
• 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: The anterior leaflet is severely restricted in
its movement and may cause significant obstruction
of the RVOT
• Type D: Almost complete atrialization of the
ventricle except for a small infundibular component.

62. Management

63. Neonatal Ebstein
• Poor prognosis
• Reported survival only 68%
• Indications for surgery-
Heart failure
Profound cyanosis

64. Initial management
• Simple observation with supplemental oxygen
• Prostaglandin infusion to maintain a PDA
• Management of pulmonary hypertension
• a) Nitric oxide b) Sildenafil
• Diuretics for CHF

66. The goals of surgery for Ebstein
anomaly are:
• 1. complete or subtotal closure of intra-atrial
communications
• 2. tricuspid valve repair or replacement
• 3. elimination of arrhythmias
• 4. selective plication of the atrialized RV
• 5. right reduction atrioplasty
• 6. repair of associated defects (VSD closure,
relief of RVOT obstruction)

67. The surgical options include-
• (a) Biventricular repair (Knott-Craig approach)
• (b) Single ventricle pathway with right
ventricular exclusion (Starnes’ approach)
• (c) Cardiac transplantation.

68. Biventricular Repair (Knott-Craig
Approach)
• TV is repaired and the atrial septum is partially
closed.
• Repair typically a mono cusp type based on a
satisfactory anterior leaflet.
• Right atrial reduction done to reduce the size of
the markedly enlarged heart to allow room for
the lungs.
• Although early mortality is high (about 25%), the
intermediate outcome appears to be promising.

69. Right Ventricular Exclusion Starnes
Approach
• Starnes et al. pioneered the right ventricular
exclusion approach, which involves:
(a) fenestrated patch closure of the TV orifce,
(b) enlarging the interatrial communication,
(c) right atrial reduction, and
(d) placing a systemic-to-pulmonary artery
shunt.

70. • Particularly useful when there is anatomic RVOT
obstruction.
• Right ventricular decompression is required as it
passively fills from thebesian venous drainage,
this is usually accomplished with a 4- to 5-mm
punch fenestration in the TV patch

71. Modified Starnes Repair (Total
Ventricular Exclusion)
• Sano et al. modified the Starnes single-ventricle
approach by performing a total right ventricular
exclusion in which the free wall of the RV is
resected and closed primarily or with a poly
tetrafluoroethylene patch .
• This simulates a large right ventricular plication,
which may improve the left ventricular filling
and provide adequate decompression to the
lungs and LV.

72. Cardiac Transplantation
• Cardiac transplantation remains an option in the
most severe forms of Ebstein’s anomaly,
particularly when there is significant left
ventricular dysfunction.

73. Children and Adults

74. Medical treatment
• Patients with Ebstein’s anomaly and cardiac failure
who are not candidates for surgery are treated with
standard heart failure therapy, including diuretics and
digoxin.
• Medical management of arrhythmias should be
individualized and combined with operative or
catheter-based intervention.
• Bacterial endocarditis prophylaxis may be required in
the presence of prosthetic materials or patches that
were used for the repair.

75. Surgery
Class I
• The following situations warrant intervention:
Limited exercise capacity (New York Heart Association class
greater than II) (Level of Evidence: B)
Increasing heart size (cardiothoracic ratio greater than 65%)
(Level of Evidence: B)
cyanosis (resting oxygen saturations < 90%) (Level of
Evidence: B)
Severe tricuspid regurgitation with symptoms (Level of
Evidence: B)
Transient ischemic attack or stroke (Level of Evidence: B)
Prior to significant LV/RV dysfunction

76. Tricuspid Valve Repair
• The goal to be achieved-
Competent tricuspid valve
Preserve RV contractility
Decrease the risk of late rhythm disturbances.

77. TV Repair
• Mayo Clinic Experience
Danielson Repair
Modified Danielson repair
• BRAZIL Experience(da Silva Approach):
cone repair;
most anatomic repair.
• FRENCH EXPERIENCE (BROUSSAIS
Approach)
• Ventriculization procedure

78. Danielson Repair
• 1979
• Creation of monocusp valve using anterior
leaflet to coapt withventricular septum
• This consisted of –
Plication of the free wall of the atrialized RV
Posterior tricuspid annuloplasty
Right reduction atrioplasty
• 30 days mortality 5.9%,survival 85% at 10
yrs,71% at 20 yrs.

79. THE BRAZIL EXPERIENCE (da SILVA
APPROACH)- Cone Reconstruction

81. • The cone technique represents the most
anatomically correct repair by completion of the
delamination process of the TV, providing 360
degrees of leaflet tissue around the AV junction
with its hinge point at the AV groove (true
annulus)

83. Relative Contraindications To The Cone
Reconstruction Technique
• Age >50 years
• Moderate pulmonary hypertension
• Significant left ventricular dysfunction: ejection
fraction <30%
• Complete failure of delamination of the septal
and posterior leaflets with poor delamination of
the anterior leaflet (<50%)
• Severe right ventricular enlargement
• Severe TV annular dilatation

84. Tricuspid Valve Replacement
• TV repair is not feasible, then porcine bioprosthetic valve
replacement remains a good alternative.
• Most prefer bioprostheses to mechanical valves due to
the relatively good durability and the lack of need for
anticoagulation
• During valve replacement in Ebstein anomaly, the suture
line is deviated to the atrial side of the atrioventricular
node and membranous septum to avoid the conduction
tissue and right coronary artery injury. This results in an
‘intra-atrial’ position of the prosthesis

86. One and Half Ventricle Repair
• The BDCPA (bidirectional cavopulmonary
shunt) does two important things in the setting
of Ebstein’s anomaly.
Reduces venous return to the enlarged,
dysfunctional RV by approx. one-third.
 Provides sufficient preload to the LV to sustain
adequate systemic perfusion when right-sided
output is low.

87. • Preferred in following situations:
(LVEDP) is <12 mmHg
the transpulmonary gradient <10 mm Hg
the mean pulmonary arterial pressure 16mm Hg

88. Indications for BDCPA
• severe RV enlargement and/or dysfunction,
• compression of the LV due to shift of the
interventricular septum,
• moderate tricuspid valve stenosis (mean
gradient >8 mm Hg) after CR
• RA to LA pressure ratio >1.5 (an indicator of
poor RV function)
• Preoperative cyanosis at rest or with exercise

89. Heart Transplantation
• Heart transplantation rarely is necessary for
Ebstein’s anomaly.
• Indication for transplantation is usually the
presence of severe biventricular dysfunction (left
ventricular ejection fraction<25%)

90. Surgical Treatment of Arrhythmias
• The most common atrial tachyarrhythmias in
Ebstein’s anomaly are atrial fibrillation and
flutter.
• biatrial maze procedure : chronic AF.
• “cavotricuspid isthmus” is also considered-in
atrial flutter

91. EP challenges in EA
• Normal hearts, the compact node is located at the apex of the
triangle of Koch.
• In EA AV node is compressed and the central fibrous body
malformed, can be closer to the orifice of the coronary sinus.
• This peculiarity extremely important trans catheter ablation of
atrioventricular nodal reentrant tachycardia
• prevalence of preexcitation in neonates with EA has been reported
to be around 10 %.
• In different series,
 5–25 % of EA patients have an accessory pathways.
 6–30 % of cases the connections are multiple

92. • right coronary artery runs in the true atrioventricular
grove,an angiography may help to identify this
anatomical landmark.
• atrialized right ventricle can be characterized by
abnormal fragmented electrograms, rendering the
interpretation of recorded signals around the target area
difficult.
• catheter manipulation is often more difficult in these
patients because of the dilatation of the right chambers. •
All these technical consideration explain lower success
rate