Tetralogy of Fallot Surgery

Overview

What is tetralogy of Fallot?

Tetralogy of Fallot is a grouping of heart defects that usually presents shortly after birth, though sometimes it’s not diagnosed for a month or two. It may even be detected before birth during a prenatal ultrasound. The word “tetralogy” means a group of four. In tetralogy of Fallot, there are four parts to the heart defects. These are:

  • Ventricular septal defect: A hole in the wall (septum) between the lower chambers of the heart.
  • Pulmonary stenosis: A narrowed or completely blocked pulmonary valve. This restricts blood flow from the heart to the lungs.
  • Malposition of the aorta: The aorta is located directly above the ventricular septal defect. This is different from a normal heart, where the aorta is completely on the left side.
  • Right ventricular hypertrophy: Thickening of the right sided pumping chamber, caused by the narrowed pulmonary valve.

What happens if my child has tetralogy of Fallot?

The problems in tetralogy of Fallot are related to the hole between the pumping chamber and the narrowed valve. Normally, the left ventricle pumps blood to the body and the right ventricle pumps it to the lungs. When you have tetralogy of Fallot, blood can flow across the ventricular septal defect (hole), from the right ventricle to the left ventricle, where it’s pumped to the rest of the body. The blood in the right side is the low oxygen blood, so the blood in the body will not have as much oxygen as it typically does.

The narrowed or blocked (obstructed) pulmonary valve also limits the blood flow to the lungs, so less blood travels to the lungs than normal.

Infants or children with tetralogy of Fallot may have a bluish tint to the skin, called cyanosis. This happens because some of the low oxygen blood that is pumped into the body across the hole (VSD). Infants or young children with tetralogy of Fallot can sometimes develop severe blue spells, called “tet spells.” During a tet spell, the baby may develop intense cyanosis (blue), often following rapid, deep breathing, increased irritability and prolonged crying. A tet spell that does not get better in a few minutes of trying to calm the baby and "break" the spell can be an emergency. Emergency medical attention is required if the tet spell persists.

Procedure Details

How is tetralogy of Fallot corrected?

Tetralogy of Fallot can be corrected with surgery. This is generally done during the first year of life, though occasionally it can be done at an older age. The goal of this surgery is to eliminate the shunting of blood across the ventricles and relieve the narrowing of the pulmonary valve to improve the blood flow to the lungs.

The complete repair involves closing the hole between the two pumping chambers with a patch, and opening up the obstruction to blood flow going to the lungs. The way in which the obstruction is opened will depend on the exact findings in each person, but can include removing some of the thickened muscle below the pulmonary valve, enlarging the valve itself — sometimes by putting a patch across it — and if needed, enlarging the branches of the arteries that go to each lung. If the valve is completely blocked, a tube (or conduit) with a valve in it may be needed to allow blood to get from the heart to the lung arteries.

Are there any other types of surgery for tetralogy of Fallot?

In some babies, a complete repair may not be optimal early in life. This might be the case for a small or premature baby, one who is very blue, or one who has other medical problems early in life. In these children, an initial operation can be performed to provide more blood to the lungs, and overcome the cyanosis. One way this can be done is by surgically placing a small tube, called a shunt, from the body artery (aorta) to the lung artery to provide more blood flow to the lungs. This does not correct the entire problem and a second, complete surgery will be needed later when the child is older. The other option that can be done soon after birth in some infants is to place a stent, or mesh tube into the patent ductus arteriosus — a natural bridging artery between the aorta (body) artery and lung artery which every baby has in the womb, but closes a few days after birth. This procedure is done in the heart cath lab, and avoids a full operation until later.

Repeat operations may be necessary in some people with tetralogy of Fallot. The surgery to open the narrowed pulmonary valve often results in leaking (regurgitation) of the valve. This leaky valve is usually well tolerated without symptoms, but can lead to either symptoms or significant enlargement of the right side of the heart in some people over time. In these cases, another surgery to replace the pulmonary valve may be needed, often in adolescence or adulthood.

Recovery and Outlook

What is the outlook of tetralogy of Fallot surgery?

The long-term outlook for children and adults with tetralogy of Fallot is good — but you will need regular follow up with a cardiologist throughout your life to monitor for heart function, heart rhythm problems and whether a later surgery for pulmonary valve replacement may be necessary. Various tests may be used to help your cardiologist monitor you, including:

  • Electrocardiograms (EKG).
  • Echocardiogram.
  • Holter monitors.
  • Exercise stress tests.
  • Cardiac MRIs.

Your cardiologist will determine if you can participate fully in sports and exercise, or whether you require any restrictions. Most people can participate in at least recreational exercise. In many cases, women with tetralogy of Fallot have had successful pregnancies, though it is important to discuss risks and recommendations with your cardiologist before getting pregnant.

Last reviewed by a Cleveland Clinic medical professional on 12/02/2020.

References

  • American Heart Association. . Accessed 12/22/2020. Tetralogy of Fallot (https://www.heart.org/en/health-topics/congenital-heart-defects/about-congenital-heart-defects/tetralogy-of-fallot)
  • Centers for Disease Control and Prevention. . Accessed 12/22/2020.Facts about Tetralogy of Fallot (https://www.cdc.gov/ncbddd/heartdefects/tetralogyoffallot.html)

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