Surgery is needed to improve blood circulation through the body and into the lungs. The most common surgeries to treat DILV are a series of two to three operations. These surgeries are similar to the ones used to treat hypoplastic left heart syndrome and tricuspid atresia.
The first surgery may be needed when the baby is only a few days old. In most cases, the baby can go home from the hospital afterward. The child will most often need to take medicines every day and be closely followed by a pediatric cardiologist. The child’s doctor will determine when the second stage of surgery should be done.
The next surgery (or first surgery, if the baby didn't need a procedure as a newborn) is called the bidirectional Glenn shunt or Hemifontan procedure. This surgery is usually done when the child is 4 - 6 months old.
After the child has had the above operations, he or she may still look blue (cyanotic). The final step is called the Fontan procedure. This surgery is most often done when the child is 18 months - 3 years old. After this final step, the baby is no longer cyanotic.
The Fontan operation does not create normal circulation in the body. However, it improves circulation enough for the child to live and grow.
A child may need more surgeries for other defects, or to extend survival while waiting for the Fontan procedure.
Your child may need to take medicines before and after surgery. These may include:
- Anticoagulants to prevent blood clotting
- ACE inhibitors to reduce blood pressure
- Digoxin to help the heart contract
For the most severe cases of DILV, a heart transplant may be recommended.