Pediatric Atrioventricular Canal Defect

  • Etiology: Spectrum of ostium primum atrial septal defect, cleft mitral leaflet, inlet ventricular septal defect, common atrioventricular valve, complete endocardial cushion defect
  • Imaging: Intracardiac left to right shunt leads to acyanosis and increased pulmonary blood flow
  • Imaging CXR: Shunt vascularity and cardiomegaly
  • Imaging Angiogram: Gooseneck deformity = elongation of left ventricular outflow tract
  • DDX:
  • Complications:
  • Treatment:
  • Clinical: 30% of atrioventricular canal defect seen in Down syndrome and 30% of Down syndrome have atrioventricular canal defect

Radiology Cases of Atrioventricular Canal Defect

CXR of AV canal / endocardial cushion defect
CXR AP and lateral shows hyperexpanded lungs, increased pulmonary vascularity and marked cardiomegaly.