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Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed
Etiology/Pathophysiology:
Complete pulmonary venous atresia leads to pulmonary venous
obstruction which results in engorged pulmonary lymphatics and
capillaries. Heart size is normal since flow through the left side of
the heart is decreased. The condition usually is fatal. It is
classified into 3 types: (1) Pulmonary lymphangiectasia associated
with congenital heart lesions characterized by obstruction of
pulmonary venous return [totally anomalous venous return below the
diaphragm, pulmonary venous atresia, mitral atresia, hypoplastic left
heart], (2) Pulmonary lymphangiectasia associated with systemic
lymphangiectasia, and (3) Isolated pulmonary lymphangiectasia. Type
(1) is the most common, Type (3) is the rarest.
Pathology:
Absence of the pulmonary venous structures, prominence of the
pulmonary lymphatics and capillaries.
Imaging Findings:
Prominent bilateral, symmetrical interstitial distention of
lymphatics with a general branching pattern radiating from the hila
that extends to the periphery with prominence of Kerley B lines.
Diffuse pulmonary hyperinflation is also
seen. Less commonly a ground glass pattern can be seen.
DDX:
References:
See References Chapter.
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