What is the treatment of Tetralogy of Fallot?

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 four defects of tetralogy of Fallot so the heart can work as normally as possible. Repairing the defects can greatly improve a child's health and quality of life.

The pediatric cardiologist and cardiac surgeon will decide the best time to do the surgery. Their decision will be based on your baby's health and weight, how severe the defects are, and how severe your baby's symptoms are.

Sometimes, teenagers or adults who had tetralogy of Fallot repaired in childhood need additional surgery to correct heart problems that develop over time.

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 passage from the right ventricle to the pulmonary artery is enlarged. These procedures improve blood flow to the lungs. This allows 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 in the septum. 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 to the lungs, it will 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 (cut) that the surgeon makes to reach the heart usually heals in about 6 weeks. The surgeon or a hospital staff member will explain when it's okay to give your baby a bath, pick him or her up under the arms, and take your baby for his or her regular shots (immunizations).

Temporary or Palliative Surgery

It was common in the past to do temporary surgery during infancy in babies who had tetralogy of Fallot. This surgery improved blood flow to the lungs. A complete repair of the four defects was done later in childhood.

Now, most babies who have 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. It also gives the baby time to grow and get strong enough for the full repair.

In the temporary surgery, 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. 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 full repair. These medicines are stopped after the shunt is removed.