Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed
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.
See related Provider Topics Congenital Heart Disease, Genetics/Birth Defects or Heart and Circulation.
See related Patient Topics Congenital Heart Disease, Genetics/Birth Defects or Heart and Circulation.
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