Hypoplastic Left Heart Syndrome Treatment

Make an Appointment

Finding out your baby has hypoplastic left heart syndrome can be devastating. Maybe this present-at-birth (congenital) heart defect showed up in an ultrasound. Or your baby had low oxygen levels after birth. Either way, it doesn’t feel like you have enough time to know what to do or where to turn.

At UVA Children’s, you'll see top heart surgeons at the #1 children’s hospital in Virginia. We have the expertise to fix the most complex heart defects. With outcomes at or better than the national average, your baby couldn’t be in better hands.

Hypoplastic Left Heart Syndrome Surgery at UVA

Because the left side of your baby’s heart didn’t develop properly, it can’t pump blood around their body. Our experts perform a series of three surgeries to create new paths for oxygen-rich blood to reach your child’s body and oxygen-poor blood to reach their lungs.

Norwood Procedure

Within several days of your baby’s birth, we’ll perform this surgery to:

  • Create a new aorta, the main artery that carries blood from your baby’s heart to their body. This new path carries oxygen-rich blood from their lower right heart chamber (right ventricle) to their body.
  • Insert a tube (shunt) to carry blood from their lower right heart chamber (right ventricle) to their pulmonary artery. 
  • Close an opening between the two major blood vessels leading away from your baby’s heart (patent ductus arteriosus),  so the new pathways will work.
  • Open the atrial septal defect, so blood can flow back to the right ventricle from the pulmonary veins.

Our outcomes for this complex surgery are higher than the national average. Babies usually stay in our hospital for less than four weeks after surgery. That’s more than a week less than the national average. We received a “very high” rating, the highest possible rating, in Norwood procedure survival from U.S. News & World Report.

Bidirectional Glenn Procedure

When your baby is 3-6 months old, we’ll disconnect the main vein that carries blood from the upper body (superior vena cava) from the heart. We’ll reconnect it directly to the artery that carries blood to the lungs (pulmonary artery).

We’ll also remove the shunt placed during the Norwood procedure. Blood returning to your baby’s heart from their upper body will go directly to their lungs. This allows the lungs to receive oxygen-poor blood and reduce the work on the heart.

Fontan Procedure

We perform this surgery when your child is about 3 years old. Children have a better outcome at this age because the blood pressure in their lung artery is lower than when they’re younger.

The surgery allows oxygen-poor blood from the lower part of their body to flow directly to their lungs. We create this path by disconnecting the main vein that carries blood from the lower body (inferior vena cava) from the heart and connecting it directly to the lungs.

Oxygen-rich and oxygen-poor blood will no longer mix in your child’s heart. Only oxygen-rich blood will flow from their lungs to their body. Your child's body will no longer look blue. 

Heart Transplant

If your child’s lower right heart chamber (right ventricle) wears out over time or the series of surgeries is not enough, they may need a heart transplant.

UVA offers the only heart transplant program for children in Virginia. U.S. News & World report rated our program “excellent,” the highest rating available.