EBSTEIN’S ANOMALY First described by Wilhelm Ebstein in 1866 ; terminology coined by Alfred...
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EBSTEIN’S ANOMALY
• First described by Wilhelm Ebstein in 1866 ; terminology coined by Alfred Arnstein
• 19 yr old cyanotic laborer with CHF
• at autopsy Ebstein found an enlarged and fenestrated ATL; STL & PTL were thickened, hypoplastic and adherent to RV. thin , dilated atrialised RV ; enlarged RA ; PFO
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< 1 % of CHD
1 per 200,000 live births
No gender preferance
Most are sporadic ; familial pattern is rare
? Genetic / reproductive / environmental risk factors
EBSTEIN’S ANOMALY
• more common in twins
• maternal exposure to benzodiazepines / lithium
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malformation of the tricuspid valve and right ventricle
characterized by
* 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);
* downward (apical) displacement of the functional annulus (septal>posterior>anterior);
* dilation of the “atrialized” portion of the right ventricle, with various degrees of hypertrophy and thinning of the wall; * redundancy, fenestrations, and tethering of the anterior leaflet
*dilation of the right atrioventricular junction (true tricuspid annulus)
Pathology
EBSTEIN’S ANOMALY
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Ebsteins Normal
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EBSTEIN’S ANOMALY
Pathology
Right ventricle
atrialised
functional
trabecular
outlet
In two third cases RV gets dilated – atrialised part RVapex RVOT
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EBSTEIN’S ANOMALY
Associated defects
interatrial communication in 80 - 94 %
rarely• bicuspid AV , subaortic stenosis, coarct , VSD, MVP
• PS , pulmonary atresia , hypoplastic pulm. arteries
Analysis of 106 cases by Jost et al (Circ.2007)
Left sided anomalies in 39 %18% of these had LV hypoplasia mimicking LV noncompaction
15 – 50 % of l – TGA has Ebstein’s like malformation of leftAV valve
AV bypass tracts in 20 - 30 %
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EBSTEIN’S ANOMALY
Classification
Carpentier et al 1988
Type A - - adequate RV volume
Type B - - large atrialised RV ; mobile ATL
Type C - - restricted mobility of ATL > RVOT obstruction
Type D - - near complete atrialisation of RV
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EBSTEIN’S ANOMALY
Physiology
RV dysfunction
Tricuspid regurgitationRetards forward flow
During atrial systole atrialised RV balloons outacting as a passive reservoir ; during ventricular systole, much of this blood is propelled back to RA
Variable – depends on the degree of pathology
High RA pressure & low LA pressure leads to R > L shuntat atrial level
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EBSTEIN’S ANOMALY
Physiology
Neonatal periodHigh PVR + RV dysfunction -- behaves like pulm. atresia
R > L shunt at atrial level ; ductus dependent
Few weeks to monthsPVR falls > improvement in RV function > fall in RA pressure> cyanosis disappears
Adolescence / adulthood RV failure > high RA pr . -- R > L interatrial shunt > cyanosis
In patients with intact IAS .. No cyanosis; but severe CHF
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EBSTEIN’S ANOMALY
Clinical features
Depends on the severity of malformaton
Asymptomatic to severely symptomatic
Cyanosis , dyspnea , palpitation , s/o CHF
Cyanosis .. Typical triphasic
Pulse .. Low volume ; arrhrythmias
JVP .. UnimpressiveElevated ; A + / V+
Precordium .. Relatively quite despite cardiomegaly RVOT pulsation+
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EBSTEIN’S ANOMALY
Clinical features
Auscultation
Cadence of sounds
• split S1 ; loud T1 ( sail sound )• split S2 ; soft P2• RV S3 , S4 • tricuspid OS
Murmurs
• TR murmur• tricuspid MDM
mimics percardial rub
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EBSTEIN’S ANOMALY
ECG
RA dilatation > tall P waves -- Himalayan P ( Taussig)
Marked RA dilatation > widening & notching of P . indicates poor prognosis
Right sided pre excitation in 20 – 30 % ( LAD of delta & QRS )
Without WPWRBBB patternQRS axis .. rightward ; rarely normal / leftwardpolyphasic QRS in precordial leadsRVH pattern .. Unusual
Arrhythmias – due to RA dilatation / WPW
SVT / A flutter / A fibVentricular arhhythmias / AV blocks .. Less common
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EBSTEIN’S ANOMALY
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EBSTEIN’S ANOMALY
Xray
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Echocardiogram
MmodeParasternal view .. Simultaneous visuslistionof mitral & tricuspid echoesMeasure the delay from mitral to tricuspid clousrenormal – 20 – 40 ms> 60 ms .. in favour of Ebsteins
2D detailed evaluation of the tricuspid valve apparatusApical displcement of septal leafletchambersassociated lesionsRV functionR > L shunt at atrial level
EBSTEIN’S ANOMALY
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EBSTEIN’S ANOMALY
MV closure
TV closureRVdilatation ; Paradoxical septal motion
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EBSTEIN’S ANOMALY
The ratio of the combined area of the right atrium andatrialized right ventricle is compared with that of the functional right ventricle and left heart ratio <0.5 grade 1 ratio of 0.5 to 0.99 grade 2 ratio of 1.0 to 1.49 grade 3 ratio ≥1.5 grade 4
Echocardiogram
Grading score .. Celemajor et al
Apical displacement of STL
Normal .. AML to STL distance is upto 8 mm / sq. m BSA.
15 mm / sq.m in children < 14 yrs 20 mm /sq.m in adults
Diagnostic of Ebstein’s
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EBSTEIN’S ANOMALY
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CMR
EBSTEIN’S ANOMALY
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EBSTEIN’S ANOMALY
Cardaic cath
Not really needed now
Mainly for EPS / RFA
Angiographic data can be obtained by CMR
Hemodynamics
Low PA and RV pressure High RA pressureSystemic desaturation
Atrialised RV … RA pressure morphology withRV electrogram
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EBSTEIN’S ANOMALY
Management
• Asymptomatic --- follow up
• Newborn with cyanosis .. Maintain PDA with PGE1
• Symptomatic
CHF .. Medical - digoxin , diuretics
Surgery
Arrhythmia .. Antiarrhtyhmics EPS > RFA
Pacing for AV block ( 4 %)
Cyanosis without significant TR ? Role of device closure of ASD
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EBSTEIN’S ANOMALY
Surgery
1959 Repair of TV in 2 pts – both died1962 First successful surgery .. TV replacement1974 Initial publication on TVR .. 54 % mortality
Indications
Methods
• Tricuspid valve replacement• Tricuspid valve repair .. Different techniques• one and a half ventricular repair• Heart transplant
• NYHA III – IV• NYHA I – II + CT ratio > 0.65• significant cyanosis• paradoxical embolism• resistant tachyarrhythmias
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Surgery
Mayo clinic series
1972 – 2005 ; 540 ptsValve reconstruction – in 35 % .. Early death – 5.4 %Valve replacement - in 65 % .. Early death - 7.6 %
Technique
• construction of a monocuspid valve using ATL• plication of free wall of atrialised RV• posterior tricuspid annuloplasty• reduction right atrioplasty
EBSTEIN’S ANOMALY
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EBSTEIN’S ANOMALY
Surgery
1988 – Carpentier et al
• longitudinal plication of atrialised RV and adjacent RA
• mobilisation of ATL and adjacent PTL and repositioning to cover the orificearea at normal level
• remodeling and reinforcement of annulus by prosthetic ring
191 cases
early mortality 9 %
mean late 20 yr survival 80%
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EBSTEIN’S ANOMALY
Surgery
One and a half ventricular repair
• tricuspid valve repair
• reduction of atrialised RV
• closure of ASD
• end to side anastamosis of SVC to RPA
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EBSTEIN’S ANOMALY
Natural history
Celemajor et al (JACC 1994 )
220 cases from 1958 to 1991 ; FU 1 – 34 yrs
Actuarial survival
67 % at 1 yr ; 59 % at 10 yrs
Predictors of death
• echo grade – 2.7 fold increase in risk for each grade increase• fetal presentation• RVOT obstruction
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