Hypoplastic Left Heart Syndrome: Treatments

How do we treat hypoplastic left heart syndrome?

At Children's Hospital Colorado Colorado Heart Institute, our doctors are experts at identifying and treating hypoplastic left heart syndrome.

Medication

After your child is diagnosed, your pediatric cardiologist will start him or her on medicine that helps keep the natural passage (ductus arteriosus) between the aorta and pulmonary artery open. This medicine is called PGE-2. It allows oxygen-rich blood and blood that needs oxygen to mix inside a child’s heart.

Surgery

There are two options for surgery for children with hypoplastic left heart syndrome: a surgical repair or a heart transplant.

Repair takes a series of surgeries, usually three, that typically take place within the first three years of a child’s life.

The first surgery, called the Norwood procedure, is performed in the first week of life. This surgery allows the right ventricle to function as the pumping chamber to get blood to both the lungs and the body.

During the Norwood procedure, the pulmonary artery and aorta are combined to create one larger path for blood to get from the heart to the body. The heart wall between the left and right atria is removed to let oxygen-rich and oxygen-poor blood mix in the heart. An artificial tube is made between the heart and the lungs, so blood can get to the lungs.

The second surgery, which is usually performed between 4 and 6 months of age, redirects blood from the upper part of the body away from the heart and directly to the lungs.

The final surgery redirects the blood from the lower part of the body away from the heart directly to the lungs. This is called the Fontan procedure and is usually performed at 2-3 years of age.

Following this series of surgeries, most children can have normal lives. But like any child with complex congenital heart abnormality, he or she will need to see a pediatric cardiologist for monitoring after the repairs.

Learn how the Complex Congenital Heart Disease and Development Clinic helps kids with hypoplastic left heart syndrome.

Heart Transplant

The second option for kids with hypoplastic left syndrome is a heart transplant. At Children’s Hospital Colorado, we have been transplanting hearts in children for more than 20 years.

Rather than doing surgery to repair the existing heart, a heart transplant will replace the defective heart with a new one from a donor. Transplant patients will require lifelong medication and will be followed by a transplant cardiologist to monitor possible rejection of the new organ.

Learn more about our nationally renowned Heart Transplant Program.

Living with hypoplastic left heart syndrome

Children with hypoplastic left heart syndrome will need regular follow-up appointments with a pediatric cardiologist to monitor his or her heart health. Most children will need to take medication after surgery.