Ebstein Anomaly: By:-Vaibhav Swarup

EBSTEIN ANOMALY
By:- Vaibhav Swarup

◤
Definition
▪ Congenital cardiac malformation characterized by apical

displacement of septal and posterior tricuspid valve leaflets
▪ Anterior leaflet is normal because formed earlier in cardiac

development
▪ Resulting in atrialization of RV with small functional right

ventricle
▪ Associated with intracardiac shunts (ASD (50%), PFO), valvular

lesions, and 25% accessory conduction pathways (WPW).
◤
◤
Characterized by
▪ (1) adherence of the septal and posterior leaflets to

the underlying myocardium (failure of delamination)
▪ (2) downward(apical) displacement of the functional
annulus (septal/posterior)
▪ (3) dilation of the “atrialized” portion of the right
ventricle, with various . Degrees of hypertrophy and
thinning of the wall.
(4) redundancy, fenestrations, and tethering of the
anterior leaflet and dilation of the right atrioventricular
junction (true tricuspid annulus)
◤
Pathologic anatomy of TV
▪ Origin of TV from AV ring & its chordal attachments are

malpositioned.
▪ Leaflets are malformed, dysplastic (thickened & distorted),

enlarged or reduced in size.
Septal leaflet always affected, posterior leaflet nearly always,
and anterior leaflet seldom.
▪ Septal & posterior leaflets displaced , maximal displacement is

usually at the commissure.
Anterior leaflet is almost always attached to AV junction. •
Adherence create characteristic displacement of valve toward
RV apex and even RVOT.
◤
Figure 1. Pathologic specimen from a
patient with Ebstein anomaly.
▪ Tricuspid valve is displaced markedly inferiorly and

the right ventricular wall is thin. With permission from
[59]. Severe right atrial (RA) enlargement, the
atrialized right ventricle (ARV), small RV, and the
apically displaced tricuspid valve (white arrow, TV)
consistent with Ebstein anomaly. A secundum atrial
septal defect (ASD) is also present.
◤
◤
Pathologic anatomy of RV
▪ • Proximal (atrialized RV)

Atrialized and dilated
When thin moves paradoxically during systole
Electrical potentials are ventricular, but
pressure pulse is atrial contoured.
◤
▪ • Distal
Smaller than normal RV
RV dilatation and dysfunction is universal
Functional portion is infundibulum, trabeculated
apex, portion beneath anterior cusp
Thinner walled with fewer muscles.
◤
◤
Etiology
▪ Congenital disease of often uncertain cause.

Most cases are sporadic, familial Ebstein’s anomaly
is rare.
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.
◤
Associated anomalies
▪ Intracardiac shunts – 50% ASD
▪ Accessory conduction pathways (WPW) – 25%

◤
Think of Ebstein’s when:
▪ Cyanotic congenital heart disease

Severe RHF
Isolated severe TR
L-transposition of great vessels
VS
Pulmonaryy stenosis or atresia with intact ventricular
septum
◤
Consequence
▪ Malformation of tricuspid valve cause TR of variable

severity
▪ Discordant contraction of atrialized RV – which

contracts with remainder of ventricle accentuating TR
▪ Can lead to right heart dysfunction/failure.

◤
Disease course
▪ Approximately 50% present with cyanosis and RHF in

infancy
▪ Remaining 50% have murmur and abnormal CXR but

initially asymptomatic
▪ Become symptomatic later in life
▪ 50 % survival at age 47
◤
Disease course post infancy
▪ •Common presenting ▪ •Uncommon presenting

symptoms: symptoms:
▪ – Dyspnea on exertion
▪ – Brain abscess from
▪ – Palpitations from R L shunt
SVT 20-30%
▪ – Bacterial
▪ – Right heart failure
endocarditis
▪ – Cyanosis, ventricular
arrhythmias ▪ – Paradoxical emboli,
stroke, TIA
◤
Management - Medical
▪ For perinatal cyanosis – wait
▪ If severe, PGE for duct patency and Nitrous Oxide to

lower pulmonary vascular resistance
▪ Persistent cyanosis in >1wk old suggests additional

pulmonary stenosis or atresia
▪ Arrhythmias: usually treated with ablation
▪ RHF in older children can be treated with digoxin or

diuretics but it is usually indication for surgical
intervention
◤
Management – Surgical
▪ Wait till pulmonary vascular resistance decreases
▪ If continuing cyanosis (O2 < 75%) treatment with

Blalock-Taussig shunt (PA to Subclavian artery)
▪ If hypoxemia still problem
▪ Glenn anastamosis – SVC to R pulmonary artery
▪ Fontan – oversew TV and close PFO so all systemic

venous return goes to pulmonary arteries and
bypasses right heart
▪ Tricuspid valve reconstruction/prosthesis – most

common.
◤
◤
Imaging Findings
▪ CXR findings
▪ –Normal
▪ –Cardiomegaly
▪ –Small aortic root and MPA shadow
▪ –Decreased pulmonary vasculature
▪ –Large RA
▪ CT and MR findings
Right sided enlargement and normal pulmonary vasculature
◤
◤

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EBSTEIN ANOMALY

By:- Vaibhav Swarup

▪ Congenital cardiac malformation characterized by apical

▪ Anterior leaflet is normal because formed earlier in cardiac

▪ Resulting in atrialization of RV with small functional right

▪ Associated with intracardiac shunts (ASD (50%), PFO), valvular

▪ (1) adherence of the septal and posterior leaflets to

▪ Origin of TV from AV ring & its chordal attachments are

▪ Leaflets are malformed, dysplastic (thickened & distorted),

▪ Septal & posterior leaflets displaced , maximal displacement is

▪ Tricuspid valve is displaced markedly inferiorly and

▪ • Proximal (atrialized RV)

▪ Congenital disease of often uncertain cause.

▪ Intracardiac shunts – 50% ASD

▪ Accessory conduction pathways (WPW) – 25%

▪ Cyanotic congenital heart disease

▪ Malformation of tricuspid valve cause TR of variable

▪ Discordant contraction of atrialized RV – which

▪ Can lead to right heart dysfunction/failure.

▪ Approximately 50% present with cyanosis and RHF in

▪ Remaining 50% have murmur and abnormal CXR but

▪ Become symptomatic later in life

▪ •Common presenting ▪ •Uncommon presenting

▪ If severe, PGE for duct patency and Nitrous Oxide to

▪ Persistent cyanosis in >1wk old suggests additional

▪ Arrhythmias: usually treated with ablation

▪ RHF in older children can be treated with digoxin or

▪ If continuing cyanosis (O2 < 75%) treatment with

▪ If hypoxemia still problem

▪ Glenn anastamosis – SVC to R pulmonary artery

▪ Fontan – oversew TV and close PFO so all systemic

▪ Tricuspid valve reconstruction/prosthesis – most

▪ –Small aortic root and MPA shadow

▪ –Decreased pulmonary vasculature

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