How Is Tetralogy of Fallot Treated?

Tetralogy of Fallot must be repaired with open-heart surgery, either soon after birth or later in infancy. The goal of surgery is to repair the defects so the heart can work as normally as possible. Achieving this goal can greatly improve a child's health and quality of life.

Your baby's heart doctor and heart surgeon will determine when to do the surgery. Their decision will be based on the baby's health and weight, how severe the defects are, and how severe the baby's symptoms are.

Sometimes, teenagers or adults who had the tetralogy of Fallot defects repaired in childhood need additional surgery to correct heart problems that develop over time. See "Living With Tetralogy of Fallot" for more information.

Types of Surgery

Complete Intracardiac Repair

Surgery to repair tetralogy of Fallot is done to improve blood flow to the lungs and to make sure that oxygen-rich and oxygen-poor blood flows to the right places. The surgeon will:

• Widen the narrowed pulmonary blood vessels. The pulmonary valve is widened or replaced and the passageway from the right ventricle to the pulmonary arteries is enlarged. These procedures improve blood flow to the lungs, allowing the blood to get enough oxygen to meet the body's needs.
• Close the ventricular septal defect (VSD). A patch is used to cover the hole. This patch stops oxygen-rich and oxygen-poor blood from mixing between the ventricles.

Fixing these two defects resolves problems caused by the other two defects. When the right ventricle no longer has to work so hard to pump blood the lungs, it can return to a normal thickness. Fixing the VSD means that only oxygen-rich blood will flow out of the left ventricle into the aorta.

The incision from the surgery usually heals in about 6 weeks. The surgeon or another member of the hospital staff will explain when it's okay to give the baby a bath, pick up the baby under the arms, and take the baby for his or her regular shots (immunizations).

Temporary or Palliative Surgery

It was common in the past to do a temporary surgery during infancy to improve blood flow to the lungs, and then do a complete repair later in childhood. Now, most babies with tetralogy of Fallot have their defects fully repaired in infancy.

However, some babies are too weak or too small to have the full repair. They must have temporary surgery first. This surgery improves oxygen levels in the blood and gives the baby time to grow and get strong enough for the full repair.

In the temporary procedure, the surgeon places a tube called a shunt between a large artery branching off the aorta and the pulmonary artery. One end of the shunt is sewn to the artery branching off the aorta, and the other end is sewn to the pulmonary artery. The shunt creates an additional pathway for blood to travel to the lungs to get oxygen. The shunt is removed when the baby's heart defects are fixed during the full repair.

After temporary surgery, your baby may need medicines to keep the shunt open while waiting for the complete repair. These medicines are stopped after the shunt is removed.