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Pediatrics C.V.S. 88a39258

Right atrial isomerization syndrome is associated with all of the following except:

A
Spleen is absent
B
Always associated with dextrocardia
C
Cyanosis is present
D
Bilateral right atria are present
High-Yield Explanation
Ans. B. Always associated with dextrocardia Ref: Nelson Textbook of Pediatrics 20th/ed p2235ExplanationDetermination of viscera atrial situs (position of atria in relation to abdominal viscera) can be made by radiography demonstration of the position of the abdominal organs and the tracheal bifurcation for recognition of the right and left bronchi and by echocardiography.# Situs solitus: The viscera are in their normal positions (stomach and spleen on the left, liver on the right), the 3-lobed right lung is on the right, and the 2-lobed left lung on the left; the right atrium is on the right, and the left atrium is on the left.# Situs inversus: The abdominal organs and lung lobation are reversed, the left atrium is on the right and the right atrium on the left.If the viscera atrial situs cannot be readily determined, a condition known as situs indeterminus or heterotaxia/isomerism.# Right isomerism or bilateral right-sidedness (asplenia syndrome) is associated with a centrally located liver, absent spleen, and 2 morphologic right lungs/right atria.# Left isomerism or bilateral left-sidedness (Polysplenia syndrome), which is associated with multiple small spleens, absence of the intrahepatic portion of the inferior vena cava, and 2 morphologic left lungs/left atria.

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