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Ebstein Anomaly (Trivia (Critical congenital heart defect, Named after…
Ebstein Anomaly
Trivia
Critical congenital heart defect
Named after William Ebstein 1866 - Joseph Prescher
Prevalence
<1%
1 in 200.000 live births
Signs and Symptoms
Atrialization of RV
Big RA
Small RV
Physical finding
S3
S4
Summation gallop
RA Hypertrophy
Right ventricular conduction defect
WPW sometimes
Related abnormalities
Anatomic abnormalities
Tricuspid
Enlargement of anterior part
Other leaflets - plastered to endocardium
Electrophysiologic abnormalities
50% have WPW --> due to atrialization of RV
Other abnormalities
Himalayan p Waves
First degree AV block
low amplitude QRS - right precordial leads
Atypical RBBB
T wave inversion in V1-V4 and Q waves in V1-V4 and II, III, aVF
Treatment
Medical
Procainamide
Contraindicated
Beta blockers
Calcium channel blocker
Digoxin
Reason: + accessory pathway
Flecainide
Propafenone
Dofetilide
Ibutilide
Surgery
Indication
NYHA III-IV
CTR>65%
Sat O2 room air <90%
Severe tricuspid regurgitation
TIA/Stroke
Displacement Tricuspid valve towards the apex of RV
Risk Factors