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endocardial cushion defect

persistence of primitive atrioventricular canal + anomalies of AV valves

associated with:

Down syndrome: in 25% of Trisomy 21 an ECD is present; in 45% of ECD Trisomy 21 is present
asplenia/polysplenia

types:

complete (AV canal)
partial

findings:

gooseneck deformity on angiogram
increased pulmonary vascularity
enlarged pulmonary artery
enlarged RV, LV, RA; normal LA (secondary to ASD)

Yong H. Hahn, MD - 2 February 1995
Last updated: 1 October 2013

Related CHORUS documents:

complete endocardial cushion defect

Down syndrome

partial endocardial cushion defect

gooseneck deformity

asplenia / polysplenia syndromes

pulmonary embolism

Copyright © 2013, Charles E. Kahn, Jr.