Hypoplastic Left Heart Syndrome

Treatment

Doctors with training in treating children with heart conditions (pediatric cardiologists) at Mayo Clinic in Minnesota treat children who have hypoplastic left heart syndrome (HLHS). A team of cardiologists, surgeons and other specialists with training in heart conditions present at birth (congenital heart disease) coordinate your child's care.

A child who has HLHS requires urgent medical evaluation once symptoms develop. Treatment of HLHS depends on the severity of your child's condition and your family's wishes. After diagnosis of HLHS, a cardiologist helps you to manage your child's condition as you decide about treatment. A cardiologist may:

• Give your child a drug to prevent closure of the connection between the left and right side of the heart (ductus arteriosus)
• Place your child on a machine that assists with breathing (ventilator)
• Give your child intravenous (IV) fluids and drugs to help his or her heart beat stronger

Doctors generally perform a three-stage surgery to treat hypoplastic left heart syndrome. Rarely, heart transplant may be an option.

Three-stage surgery

A three-stage surgery is an option for some children who have HLHS. In this operation, surgeons reconstruct your child's heart so that the single pumping chamber can meet your child's needs for the rest of his or her life. Completion of these operations allows separation of the blue blood (blood without oxygen) from the red blood (blood with oxygen).

• Stage one operation. Doctors perform the first stage, the Norwood procedure, in your child's first week of life. In this procedure, doctors reconstruct the main blood vessel supplying blood to the body (the aortic arch) and insert a tube (shunt) connecting the aorta to the blood vessel supplying the lungs (the pulmonary artery).
  
  

  Mayo Clinic surgeons also perform a newer form of this operation, connecting the lower-right heart chamber (right ventricle) to the pulmonary artery. This procedure allows the lower-right heart chamber (right ventricle) to pump blood to the child's lungs and body.

  The Norwood procedure needs to be performed soon after your child is diagnosed with HLHS. Your child will still look blue (cyanotic), after this first-stage operation.

• Stage two operation. Doctors perform the second operation, the bidirectional Glenn procedure or hemi-Fontan, when your child is four to six months old. In this procedure, they connect some of the veins carrying blood from the body to blood vessels carrying blood to the lungs.
  
  

  This surgery allows most of the blood to flow directly from the body into the lungs. Blood with more oxygen is pumped to the aorta to supply oxygen to the body's organs and tissues. This approach reduces the work of the lower-right heart chamber (right ventricle) by allowing it to pump blood only to the body.

• Stage three operation. Doctors perform the third surgery, the Fontan procedure, when your child is 18 to 48 months. In the Fontan procedure, the remaining blood vessels carrying blood from the body are connected to the blood vessels carrying blood to the lungs.
  
  

  This surgery allows the rest of the blood coming back from the body to go to the lungs. The blood with oxygen and the blood without oxygen no longer mix in your child's heart. After this operation, your child no longer looks blue. With more oxygen-rich blood going to the body, your child's general health and growth improves.

Follow-up care

Your child will need long-term monitoring of the reconstructed heart and blood vessels. Your child may need to take heart medications. Follow-up appointments include echocardiograms and heart catheterization to test heart function as your child grows.