- 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
- Cleft AV Valve
- 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”
- 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
- 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)