Treatment

All infants and children with tetralogy of Fallot must have surgery to repair the defects or help improve their symptoms.

The goals of treatment are to:

• Improve the baby's symptoms
• Increase the level of oxygen in the baby's blood
• Repair the defects

Most infants have surgery by the time they are 6 months old. There are different types of surgery for tetralogy of Fallot. Your baby's heart doctor and heart surgeon will determine the type of surgery needed and when it should be performed. Their decision will be based on:

• The baby's health and weight
• Severity of the baby's defects
• Severity of the baby's symptoms

Sometimes, teenagers or adults also need surgery to correct continued problems.

Types of Surgery

Corrective surgery

Surgery to repair the defects of tetralogy of Fallot involves:

• Closing the ventricular septal defect (VSD)—the hole in the inner wall of the heart between the lower chambers. A patch is used to cover the hole. This cover stops the mixing of blood between the chambers. The oxygen-rich blood now flows out of the heart only to the body, and the oxygen-poor blood goes to the lungs.
• Opening and enlarging the area that blood flows through as it leaves the lower right side of the heart. The thickened heart muscle is opened, or a small amount of heart muscle is removed. This improves the flow of oxygen-poor blood to the lungs so that it can pick up more oxygen.
• Opening or widening the pulmonary valve (between the right ventricle and the pulmonary artery). The valve can be opened using a special instrument, but often a patch is sewn on the heart to make the narrow area bigger. This increases blood flow out of the heart to the lungs.

Temporary or palliative surgery

Some babies are too weak to have open-heart, corrective surgery. They have temporary surgery, which does not repair the defects of tetralogy of Fallot, but partially improves oxygen levels in the blood to give the baby time to grow and get stronger so the problem can be fixed later.

Instead of open-heart surgery, a small opening can be made between the ribs. The procedure involves:

• Placing 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 pulmonary artery, and the other end is sewn to an artery branching off the aorta. This creates an additional pathway for blood to travel to the lungs.
• This new pathway allows some of the blood in the aorta to flow through the tube into the pulmonary artery, where it travels to the lungs to pick up oxygen.
• The shunt is removed when your baby's heart defects are repaired during the corrective surgery.

After surgery, your baby may need medicines to help keep the new blood pathway open.

Treatments While Waiting for Surgery

Your baby may need other treatments before surgery. These treatments help the baby get stronger. They include:

Nutrition

Babies with tetralogy of Fallot can tire while nursing or feeding. You may need to feed your baby more often. Some babies also need extra nutrition. This is given as a supplement or an extra feeding. These feedings usually are formulas that give the baby extra calories. Many babies need extra vitamins or iron. Your child’s doctors will decide what extra nutrition your baby might need.

Lowering stress

Lowering your baby's anxiety or stress can help prevent tetralogy "spells" and save the baby's energy. You can try to predict what your baby needs to stop or to lessen crying. For example, picking up an infant slowly and speaking in a soothing voice can avoid startling a baby.

Treating tetralogy spells

If your baby has a tetralogy spell, you should:

• Bring the baby's knees up tight against the baby's chest (the knee-chest position).
• Attempt to calm the baby.
• Call 911 if your baby's symptoms do not immediately improve.

Even if the baby's symptoms improve at home without emergency treatment, you should still contact your doctor to report the spell.

After Surgery

After temporary surgery, your baby may need medicines to keep the shunt open while waiting for the corrective surgery. These medicines are stopped after the shunt is removed during the corrective surgery. It is rare to need medicines after corrective surgery unless there are other problems.

The scar from surgery usually heals in about 6 weeks. Your surgeon or another member of the hospital staff will tell you when:

• You can give your baby a bath
• You can pick the baby up under the arms
• Your baby should get his or her regular shots (immunizations)

Your baby may need to take medicines to prevent a serious respiratory infection called respiratory syncytial virus (RSV). Because of having the heart defect, your baby may be more prone to get this infection. Your pediatrician or family doctor will decide if your baby needs any medicines to prevent RSV.

Reference:

National Heart, Lung, and Blood Institute, USA.