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)

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© 2002, C.E. Kahn, Jr.
2002-04-18