Treatment

All babies who have tetralogy of Fallot need corrective surgery performed by a heart (cardiovascular) surgeon. Without treatment, your baby might not grow and develop properly. Your doctor will determine the most appropriate surgery and the timing of the surgery based on your or your child's condition.

Some children may need medicine while waiting for surgery to maintain blood flow from the heart to the lungs.

Surgery or other procedures

Surgery for tetralogy of Fallot involves open-heart surgery to correct the defects (intracardiac repair) or a temporary procedure that uses a shunt. Most babies and older children have intracardiac repair.

Intracardiac repair

This open-heart surgery is usually done during the first year after birth and involves several repairs. Adults with tetralogy of Fallot rarely may undergo this procedure if they didn't have surgical repair as children.

During intracardiac repair, the surgeon will:

• Patch over the ventricular septal defect to close the hole between the lower chambers of the heart (ventricles).
• Repair or replace the narrowed pulmonary valve to increase blood flow to the lungs.

Because the right ventricle won't need to work as hard to pump blood after this procedure, the right ventricle wall will go back to its normal thickness. After intracardiac repair, the oxygen level in the blood increases and symptoms decrease.

Temporary shunt surgery

Occasionally babies need to undergo a temporary (palliative) surgery before having intracardiac repair in order to improve blood flow to the lungs. This procedure may be done if your baby was born prematurely or has pulmonary arteries that are undeveloped (hypoplastic).

In this procedure, the surgeon creates a bypass (shunt) between a large artery that branches off from the aorta and the pulmonary artery.

When your baby is ready for intracardiac repair, the surgeon removes the shunt during the procedure for intracardiac repair.

After surgery

The long-term survival rates for people who've had tetralogy of Fallot surgery continue to improve.

However, sometimes blood flow to the lungs may still be restricted after tetralogy of Fallot surgery. Additional surgeries may be needed. An adult with repaired tetralogy of Fallot may have a leaky pulmonary valve (pulmonary valve regurgitation) and may need to have their pulmonary valve eventually replaced.

Heart rhythm problems (arrhythmias) are common after tetralogy of Fallot repair surgery. Your doctor may recommend medications, a procedure to treat the arrhythmias (ablation) or a special pacemaker that treats life-threatening arrhythmias (implantable cardioverter-defibrillator).

Ongoing care

People with tetralogy of Fallot need lifelong care with a pediatric or adult congenital cardiologist to ensure the surgery's success and to monitor for complications. Checkups often include imaging tests to determine how well treatment is working.