Once the diagnosis is made, the baby will often be admitted to the neonatal intensive care unit (NICU). A medicine called prostaglandin E1 may be used to keep the ductus arteriosis open so that blood can circulate to the lungs.
The condition always requires surgery. If the heart is unable to pump enough blood out to the lungs and rest of the body, the first surgery usually occurs within the first few days of life. In this procedure, an artificial shunt is inserted to keep blood flowing to the lungs. In some cases, this first surgery is not needed.
Afterward, the baby usually goes home. The child will need to take one or more daily medicines and be closely followed by a pediatric cardiologist, who will decide when the second stage of surgery should be done.
The next stage of surgery is called the Glenn shunt or Hemifontan procedure. This procedure connects half of the veins carrying blue blood from the upper half of the body directly to the pulmonary artery. The surgery is usually done when the child is between 4 to 6 months old.
During stage I and II, the child may still look blue (cyanotic).
Stage III, the final step, is called the Fontan procedure. The rest of the veins carrying blue blood from the body are connected directly to the pulmonary artery leading to the lungs. The left ventricle now only has to pump to the body, not the lungs. This surgery is usually performed when the child is 18 months to 3 years old. After this final step, the baby is no longer blue.