What is a Fenestrated Fontan?
Fontan surgery is done on children who are born with HLHS (Hypo-plastic Left Heart Syndrome), a condition where all the structures on the left side of the heart are severely underdeveloped. The left side of the heart receives oxygen-rich blood from the lungs and pumps it out to the body. When this side is underdeveloped, it is completely unable to support circulation needed by the body’s organs.
The Fontan surgery is done in stages, attempting to reconfigure the child’s cardiovascular system despite the lack of a working left ventricle. The first operation, typically performed in the first week of life, involves reconstruction of the aorta and pulmonary arteries so that the flow of blood from the right ventricle is directed primarily to the remodeled aorta. To accommodate the change in pressure that increases to maintain blood flow, a hole (fenestration) is left in the fabric patch used to create a tunnel. Through this hole, the tunnel could decompress into the left atrium whenever the pressure in the tunnel becomes dangerously high. After time passes and the child’s condition stabilizes, the hole can be closed using the Cardia device.