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Virtual Pediatric Hospital: Paediapaedia: Ebstein Anomaly Paediapaedia: Cardiovascular Diseases

Ebstein Anomaly

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Can present at any age with cyanosis. There may be chest pain or palpitations related to cardiac arrythmia. Life expectany is 10-20 yrs with death due to right sided heart failure.

Etiology/Pathophysiology:
Is due to a low insertion of the tricuspid valve which divides the right ventricle into proximal and distal chambers. The proximal atrialized division works as a receiving chamber with the right atrium. The more distal ventricular division acts as pumping chamber. A functional obstruction at the level of the tricuspid valve exists due to asynergous diastole of atrium during systole of the atrialized right ventricle. The tricuspid valve is often deformed, leading to tricuspid regurgitation. A right to left interatrial shunt is often present. The clinical course depends on the amount of tricuspid valve deformation / obstruction and the amount of right to left shunt.

Pathology:
Not applicable

Imaging Findings:
On the CXR cardiomegaly with a prominent right heart border and decreased pulmonary vasculature is seen. A "box like" configuration to the heart can be seen in older children due to a combination of right atrial enlargement and outward displacement of the right ventricular outflow tract.

DDX:

References:
See References Chapter.

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