How is double inlet left ventricle treated?

Double inlet left ventricle is treated with surgery to repair the defect. Several surgeries may be needed in order to make the heart work effectively. In most cases, the heart can be repaired to the point where the child can lead a relatively normal life.

With surgery, blood circulation through the body and into the lungs can be improved. Your baby may have a number of surgeries in order to have a surgery called the Fontan procedure.

If blood flow to the pulmonary artery is restricted, a small tube called a shunt can be surgically placed from an artery attached to the aorta to the pulmonary artery. If there is too much blood flow to the lungs, the surgeon may perform a pulmonary artery banding, which protects the pulmonary arteries from high blood pressure.

The first surgery may have to be performed within the first few days of your baby’s life. Your baby usually goes home after the first surgery. A pediatric cardiologist will decide when your child should undergo the second surgery. The follow-up surgery (or initial surgery if your baby was not operated on in the first few days of life) is called a Glenn shunt procedure. This operation usually is performed when your baby is 4 to 6 months old.

The final procedure is the Fontan procedure and usually is done when your child is 3 to 6 years old. After the final stage of surgery, the baby’s oxygen levels will be similar to a child with a normal heart. Although the Fontan procedure doesn’t result in normal blood circulation in the body, it does improve the circulation and allows your child to grow.

Your child may need additional types of surgeries for related defects or to extend survival while waiting for the Fontan procedure. The Fontan procedure has risks and complications, which your child’s doctor will discuss with you.

Your child’s doctor may prescribe medication before and after surgery. These medications may include:

  • Digoxin to help with heart contractions.
  • ACE inhibitors to lower blood pressure.
  • Anticoagulants to prevent blood clots.
  • Diuretics to remove extra fluid from the body.

In addition, children with double inlet left ventricle have to take antibiotics before dental treatment in order to prevent endocarditis (infection of the heart lining).

In the most severe cases of double inlet left ventricle, a surgeon may recommend a heart transplant for your child.

Last reviewed by a Cleveland Clinic medical professional on 10/28/2020.

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