Hypoplastic Left Heart Syndrome

Treatment

Pediatric specialists at Mayo Clinic Rochester have extensive experience treating children with HLHS. Patients' care is coordinated through a team of specialists from the Center for Congenital Heart Disease. Children needing hospitalization are cared for at Mayo Eugenio Litta Children's Hospital in Rochester.

Treatment Options

Managing this condition depends on the family's wishes and the severity of the condition. Once the diagnosis is made, a number of things are done. First, a medicine called prostaglandin is started to prevent the ductus arteriosus from closing. A ventilator may be used to help with breathing. Intravenous fluids are started and medicines to help the heart beat stronger often are used. This will help stabilize the baby while decisions are made regarding treatment.

Three-stage surgical procedure

A three-stage surgical procedure is an option for some babies. The operation reconstructs the child's heart so that the single pumping chamber can meet the patient's needs for the rest of his or her life. The first operation is performed in the first week of life The second operation is performed around 6 months and the third operation is performed when the child is 18 to 48 months. Completion of these operations allows separation of the blue blood (blood without oxygen) from the red blood (blood with oxygen).

• Stage one operation
  The first stage is called the Norwood Procedure. Because the left ventricle cannot pump blood adequately to the body, the Norwood procedure allows the right ventricle to pump blood to both the lungs and the body. The procedure involves reconstructing the aortic arch (the main blood vessel supplying blood to the body) and inserting a tube that connects the aorta to the pulmonary artery (the blood vessel which supplies blood to the lungs). This operation needs to be done soon after the diagnosis is made. The baby will still look blue (cyanotic), after this first-stage operation.
• Stage two operation
  The second operation, called the bidirectional Glenn procedure or hemi-Fontan, reduces the work of the right ventricle by allowing it to pump blood only to the body, and allowing most of the blood to flow directly from the body into the lungs. After this operation is performed, all the blood coming from the upper body is sent to the lungs so more blood with oxygen is coming back from the lungs to the left side of the heart.
• Stage three operation
  The third and final operation, called the Fontan procedure, allows the rest of the blood coming back from the body to go to the lungs. Now there is no mixing of blood with oxygen and blood without oxygen in the child's heart. After this operation, the child will no longer look blue. With more oxygen-rich blood going to the body, the child's general health and growth will improve.

Heart transplant

Babies with HLHS can be put on a list to receive a heart transplant. When the baby's name is at the top of the list, he or she will receive a healthy donor heart. To fight against rejection of the new heart, transplant patients need to take anti-rejection medications for the rest of their lives. The baby is kept on prostaglandin until a donor heart becomes available, and then a transplant has to be performed at a center like Mayo Clinic which is staffed and equipped to perform heart transplants in newborn infants.

Follow up

Long-term follow-up is needed to monitor the function of the reconstructed heart and blood vessels. The infant/child may need ongoing treatment with cardiac medications. During office visits the function of the child's heart can be monitored with echocardiography, an ultrasound to check the heart pump function and the blood vessel as the child grows and develops. Two heart catheterizations are typically performed over the first 2 years, usually just before each of the next 2 surgical stages, after the initial Norwood procedure.

In a heart catheterization, soft, thin plastic tubes (catheters) are placed in the large blood vessels in the leg and threaded carefully to the heart. The catheters are used to take pressure measurements inside the heart and to inject contrast or dye so pictures of the heart can be taken. Overall, this is a very safe test and children can go home the same day.