Complete AV Septal Defect


  • Partial AV Canal Defect –> aka Primum ASD
  • Complete AV Canal Defect 
    • ASD above the valve
    • VSD below the valve
    • Instead of 2 valves –> One large AV valve


Comparison of normal and complete AVSD anatomy. (CDC Public Domain)


Complicating Features

  1. Cleft AV Valve
  2. LVOT Obstruction (“Gooseneck Deformity”)
    • Aortic valve positioned anteriorly and farther from the apex than the mitral valve (normally equidistant from apex).
    • Long LVOT can obstruct, this is known as the “Gooseneck Deformity”
  3. AV Block


  • Usually associated with a massive shunt that needs repair in early childhood.
  • Without intervention, the large shut causes increase in pulmonary flow, which raises pulmonary vascular resistance causing Eisenmenger syndrome.
  • A PA-band may be used to protect the pulmonary circulation from rising vascular resistance until definitive repair

Surgical Repair

  • Complete repair can be attempted to separate the AV valves, atria and ventricles
  • If RV is not well developed enough –> Single ventricle palliation will need to be performed (Bidirectional Glenn –> Fontan)

Case #2