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Sequence of presentation
Anatomy
Work up
Treatment
Embryology
Clinical Features
TV annulus
The tricuspid valve is the most apically (or
Leaflets
The tricuspid valve has three distinct
larger.
The posterior leaflet is smaller and appears
Leaflets
The septal leaflet is in immediate proximity
Transthoracic Views
Transesophageal Views
Embryologic aspect
The leaflets and tensile apparatus of the atrio ventricular
DEFINITION
Congenital defect
Origins of septal or posterior
sail-like
Pathologic anatomy of TV
Origin of TV from AV ring & its
displaced , maximal
displacement is usually at the
commissure
Pathologic
anatomy of RV
Proximal
Atrialized in one fourth of hearts\dilated
When thin moves paradoxically during systole
Electrical potentials are ventricular, but pressure pulse
is atrial contoured
Distal
Smaller than normal RV
RV dilatation
Functional portion is infundibulum, trabeculated apex,
Severe Ebsteins
malformation of
tricuspid valve (4chamber view) showing
marked downward
displacement of shelflike posterior leaflet
with attachment to
underlying free wall by
numerous muscular
stumps (arrows),
markedly dilated
atrialized portion of
right ventricle (ARV),
small functional portion
of right ventricle (RV),
leftward bowing of
ventricular septum, and
marked dilatation of
right atrium (RA). LA
indicates left atrium; LV,
left ventricle
Epidemiology
Ebsteins anomaly occurs in 0.3-0.8% of all
Associated defects
Commonly associated with:
ASD or PFO (90%)
VSD, AV canal defect
Pulmonary stenosis/atresia (20-25%)
Wolff-Parkinson-White
Syndromes:
Down, Marfan, Noonan
Etiology
Congenital disease of often
uncertain cause.
Environmental factors
Maternal ingestion of lithium in first
trimester
Maternal benzodiazepine use
Maternal exposure to varnishing
substances
Maternal history of previous fetal
loss
Risk is higher in whites than in
other races.
Genetic factors
Rare cases of cardiac transcription factor NKX2.5
mutations, 10p13-p14 deletion, and 1p34.3p36.11 deletion have been described in the
anomaly .
Recently, Postma et al. reported the results of a
mutational analysis in a cohort of 141 unrelated
probands with Ebstein anomaly.
Eight were found to have a mutation in the gene
MYH7 and six of the eight patients also had left
ventricular noncompaction.
This may warrant genetic testing and family
evaluation in this subset
Origin
1866 A.D.
Hypothesis on single autopsy specimen
He even did not see the patient, when later
was alive
Word used in 1927 A.D.
A pencil
sketch of
Wilhelm
Ebstein
published in
the
Festschrift
celebrating
Ebsteins 70th
birthday.
Published by
permission of
the Mayo
Clinic
Proceedings,
where it was
published by
Mann RJ, Lie
JT.
The life story
of Wilhelm
Ebstein (1836
1912) and his
Clinical presentation
echocardiography.
Neonatal life and infancy:
Cyanosis and/or severe
heart failure
Improve as pulmonary
vascular resistance
decreases.
Adult life:
Fatigue, exertional dyspnea,
cyanosis, tricuspid
regurgitation and/or right
heart failure, and
palpitations; arrhythmias
are common.
Neonatal presentation
Congestive heart failure
Due to TR and RV dysfunction
Cyanosis
Decreased pulmonary blood flow due to R
Later presentation
Cyanosis
Due to R L shunt at atrial level
ejection fraction
Palpitations and sudden cardiac death
Incidental murmur
Paradoxical embolism
Cyanosis
Fairly common
Right-to-left shunt at the atrial level and/or severe heart
failure
Transient in neonatal life with recurrence in adult life
May appear for the first time in adult life
Transient appearance/worsening of cyanosis in adult life
death
SVT in as many as one third of patients
Arrhythmias
Due to right atrial enlargement and high
Electrophysiologic studies
25-30% have accessory pathways
5-25% have evidence of preexcitation on the
surface ECG.
Right-sided pathways are more common.
Fifty percent of the patients have multiple
pathways.
Guide ablative therapy.
Complications
Congestive heart failure
Sudden cardiac death
Bacterial endocarditis
Brain abscess
Paradoxical embolism
Transient ischemic attacks
Stroke
adequate supervision.
Prenatal presentation
Difficult to diagnose prenatally
Fetal presentation is variable: possible
Physical Examination
Cyanosis and clubbing - Varying degrees of
Physical Examination
Arterial pulses
Usually normal
Diminished volume
Heart sounds
First heart sound
widely split with loud tricuspid component
Mitral component may be soft or absent in the presence of
prolonged PR interval.
Second heart sound
usually is normal
widely split when the pulmonary component is delayed due to
RBBB.
Physical Examination
Additional heart sounds and murmurs
Third and fourth heart sounds
commonly present, even in the absence of congestive heart
failure (CHF).
Summation of third and fourth heart sounds, especially with
prolonged PR interval, can mimic an early diastolic murmur.
the apex
Murmur intensity and duration increase during
inspiration.
Routine investigation
protocal
12-lead ECG
Rhythm
Usually normal sinus findings
Intermittently SVT, paroxysmal
Himalayan P waves
PR interval
Most commonly prolonged
May be normal or short in
patients with WPW (B) syndrome
QRS complex
RBBB
Chest XRay
Cardiomegaly
( Rounded or Box-like
contour )
Small aortic root
and main
pulmonary artery
shadow
Decreased
pulmonary
vasculature
Large right atrium
Echocardiogram
Standard for diagnosis.
M-mode
Paradoxical septal motion
Dilated right ventricle
Delayed closure of tricuspid valve leaflets
Echocardiogram
Two-dimensional
Apical displacement of the septal leaflet of
contractile performance
Various left heart structural abnormalities
Echocardiogram
Doppler studies
Varying degrees of tricuspid regurgitation
Excludes associated shunts
Echocardiogram
Assessment of severity and surgical options
Functional right ventricular area less than 35% of total right ventricular area or an
atrialized to functional right ventricular ratio greater than 0.5 associated with
unfavorable prognosis
Functional right ventricular size
Degree of septal leaflet displacement
Amount of leaflet tethering
Magnitude of leaflet deformity and dysplasia
Aneurysmal dilatation of right ventricular outflow tract (right ventricular outflow
Cardiac MRI
CMR imaging has emerged as another tool
Cardiac Catheterization
No longer required to make/confirm the
diagnosis
The most diagnostic characteristicTypical atrial pressure & ventricular
intracardiac ECG in the atrialized portion of
the RV
Elevated RAP
R-L atrial shunting with systemic
desaturation
Elevated RVEDP
Natural history
Natural history
It is not uncommon for Ebsteins anomaly to be
Natural history
Predictors of cardiac-related death on univariate
analysis included:
Cardiothoracic ratio of 0.65
Increasing severity of TV displacement on
echocardiography
New York Heart Association (NYHA) class III or IV
Cyanosis
Severe TR
Younger age at diagnosis
In a multivariate model, younger age at diagnosis, male
sex, cardiothoracic ratio of 0.65, and the severity of
TV leaflet displacement on echocardiography were
predictors of late cardiac mortality.
Management
Severity Assessment
Guideline/Indications
Medical management
Surgical management
Post operative functional status
Severity assessment
Celermajer Index
Celermajer et al. described an echocardiographic
GOSE score
Grade
1
2
3
Ratio
<0.5
0.50.99
1-1.49
Mortality
8%
9%
1-1.49
100%
>1.5
100%
(acyanoti
c)
10% (neonatal)
45% (later)
(cyanotic
)
Carpentiers classification
In 1988, Carpentier et al. proposed the
EBSTEINS ANOMALY
(Carpentiers classification)
TYPE (M- C -)
M mobility
C - contractility
TYPE B (M+ C -)
CANADIAN CARDIOVASCULAR
SOCIETY
CANADIAN CARDIOVASCULAR SOCIETY
2009 CONSENSUS CONFERENCE
UPDATE ON THE GUIDELINES FOR
THE MANAGEMENT OF ADULTS WITH
CONGENITAL HEART DISEASE
EBSTEIN ANOMALY
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)
Important 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)
Patients who require operation for Ebstein anomaly should be
operated on by congenital heart surgeons who have
substantial specific experience and success with this
operation. Every effort should be made to preserve the
native TV. (Level of Evidence: C)
Presentation at Annual CCS Meeting in Edmonton 2009
Neonatal Ebstein
Neonatal Ebstein
Poor prognosis
Reported survival only 68%
Indiactions for surgury Heart failure
Profound cyanosis
Initial management
Prostaglandin infusion
Placement of umbilical catheters
Initiation of mechanical ventilation
Minimum possible mean airway pressure
Tidal volumes of 10-15 ml/kg to overcome
atelectasis
Management of pulmonary hypertension
Diuretics for CHF
Management of pulmonary
hypertension
Nitric oxide
Reduces after load of right ventricle
Sedation
Other pulmonary vasodilators
partially closed.
This repair is typically a mono cusp type
based on a satisfactory anterior leaflet
Routine right atrial reduction is important
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.
Survival to hospital dismissal was 74% with
no late
Ann Thorac Surg 2002;73:17861
Cardiac Transplantation
With the improved results of the
Surgery
INDICATIONS FOR SURGERY
Presence of symptoms,
Cyanosis,
Paradoxical embolization.
Patients who have decreased exercise performance
Progressive increase in cardiothoracic ratio
Progressive right ventricular dilatation and
dysfunction
Onset or progression of arrhythmias
Presence of class III or IV NYHA or significant
symptoms
surgery:
(a) Closure of any intra cardiac
communications
(b) TV repair or replacement
(c) Ablation of arrhythmias
(d) Selective plication of the atrialized RV
from apex to base,
(e) Reduction right atrioplasty
(f) Repair of associated defects (e.g.,
closure VSD).
Danielson Repair
This repair technique was reported in 1979
THE VENTRICULIZATION
PROCEDURE
Ullmann et al. published their results with
Tricuspid Valve
Replacement
Every effort should be made to repair the
Heart Transplantation
Heart transplantation rarely is necessary
Arrythmia management
The most common atrial tachyarrhythmias in