Tetralogy of Fallot

Duke’s team of congenital heart disease specialists provide compassionate and comprehensive care for children and adults born with tetralogy of Fallot. We are one of the few centers in North Carolina with the experience and expertise needed to surgically repair tetralogy of Fallot. We carefully consider each person’s condition and offer the full range of treatment options, including minimally invasive catheterization procedures for those who qualify.

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About Tetralogy of Fallot

Tetralogy of Fallot is a combination of structural problems that develop in the heart before birth. Together, these four congenital heart defects allow oxygen-rich and oxygen-poor blood to mix, which can lead to high pressure in the heart and reduced oxygen to the body. The four conditions that comprise tetralogy of Fallot are:

  • Ventricular septal defect (VSD): a hole between the bottom two chambers of the heart.
  • Overriding aorta: your body’s main artery connects to both of the heart’s lower chambers instead of just the left chamber.
  • Pulmonary artery stenosis: constriction in the artery that transports blood from the heart to the lungs.
  • Right ventricular hypertrophy: the muscle in the lower right heart chamber thickens as it works harder to compensate for other heart abnormalities.

 

Disease Spectrum
Tetralogy of Fallot ranges in severity. The following subtypes of Tetralogy of Fallot are especially rare and severe: 

  • Tetralogy of Fallot with pulmonary atresia: The pulmonary valve is missing and sealed shut. This can lead to undersized pulmonary arteries and require intervention as a baby.
  • Tetralogy of Fallot with major aortopulmonary collaterals: Pulmonary atresia prompts the formation of new blood vessels to supply blood to the lungs. This can require multiple surgeries to promote pulmonary artery growth.
  • Tetralogy of Fallot with absent pulmonary valve: The pulmonary valve leaflets do not develop, which can result in significant dilation of the pulmonary arteries and may cause airway concerns.
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Tests and Diagnosis

Tetralogy of Fallot is usually discovered before birth during a routine prenatal ultrasound and confirmed with a specialized fetal echocardiogram. After birth, tetralogy of Fallot is typically diagnosed before a child’s first birthday due to symptoms that include a heart murmur, blue or gray skin color, trouble breathing, losing consciousness, slow weight gain, fatigue, or unexplained irritability. “Tet spells” are episodes when symptoms manifest during times of exertion, like feeding or crying.

The following tests can help identify tetralogy of Fallot:

Pulse Oximetry
A small sensor clipped on a finger measures the amount of oxygen in the blood.

Echocardiogram
This special ultrasound of the heart shows its structure and function.

Chest X-ray
X-ray images show the location of dense chest structures like the heart, lungs, and ribs.

Cardiac CT
A cardiac computed tomography (CT) scan creates 3D and 4D images of the heart.

Electrocardiogram
Small electrodes placed on the chest record the heart’s electrical activity.

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Tetralogy of Fallot Treatment

Tetralogy of Fallot almost always requires surgical repair, often within the first six months of life. Doctors will consider your child’s age, size, oxygen levels, and individual anatomy when recommending a surgical plan. If your child is stable, delaying open-heart surgery reduces the risk of complications, shortens hospital stay, and results in better outcomes.

Complete Repair: Tetralogy of Fallot Repair Surgery

Tetralogy of Fallot repair is an open-heart surgery that requires breaking the chest bone (this is called a sternotomy) and use of a heart-lung bypass machine (also known as cardiopulmonary bypass). During this all-in-one surgery, pediatric heart surgeons close the ventricular septal defect and widen the connection between the heart and the pulmonary artery, restoring proper oxygenation and blood flow. This connection can be made through several surgical approaches, depending on your child’s anatomy.

Valve Replacement in Infants
Babies with a severely malformed or missing pulmonary valve will also need their valve replaced during their lifetime. Timing and options for valve replacement depend on your child’s type of defect, age, and size.

Your child may be eligible for valve-sparing surgery, which uses a child’s own tissue to build a functioning pulmonary valve. This means the valve grows with them, potentially eliminating the need for valve replacement surgery later in life.

A partial heart transplantation, also known as a living heart valve replacement, is another potential option. It uses a healthy valve from a donor heart to replace a defective valve. Like valve-sparing surgery, the transplanted valve grows with your child.

Temporary Treatment and Partial Repairs

To stabilize heart function until tetralogy of Fallot repair surgery can be performed, newborns may require one or more of the following treatments to improve oxygenation and blood flow in the meantime.

  • Medication: Prostaglandin keeps the ductus arteriosus open to improve blood flow to the lungs. This passageway, which normally closes on its own after birth, connects the pulmonary artery and aorta.
  • Catheterization Procedures: During minimally invasive catheterization procedures, interventional cardiologists can make heart repairs by inserting a small, flexible tube called a catheter into a blood vessel (usually in the groin) and guiding it to the heart. They pass small tools through the catheter to place the following stents (metal mesh tubes):
    • A ductal stent may be placed in the ductus arteriosus to keep it open. This allows babies to stop taking prostaglandin medicine, which can have undesirable side effects.
    • A right ventricular outflow tract (RVOT) stent may be placed across the pulmonary valve and muscle bundles to hold them open.

Adolescent and Adult Valve-Replacement Surgery

As adolescence nears and/or in adulthood, you may need to have your pulmonary valve replaced, either for the first time, or again if you had valve-replacement surgery as a child. Duke offers several approaches to valve-replacement surgery, including traditional open-heart surgery, a less-invasive approach in which surgeons access the heart through a small incision between two ribs, or a minimally invasive catheterization approach that requires only a small incision in the groin.

Adult Care for Tetralogy of Fallot

Thanks to advancements in pediatric heart surgery, people born with tetralogy of Fallot are living long and healthy lives. However, if you were born with tetralogy of Fallot, even if you aren’t experiencing any obvious heart-related symptoms, it’s important to see an adult congenital heart specialist regularly to monitor for potential issues. At Duke, children with congenital heart defects usually start transitioning to adult care between the ages of 18 to 24. 

#3 in Nation and #1 in NC for Pediatric Cardiology and Heart Surgery

Duke Children’s is ranked the #3 pediatric cardiology program in the nation and the best in North Carolina by U.S. News & World Report.

Why Choose Duke

Proven Outcomes
Duke’s pediatric tetralogy of Fallot surgery survival rates exceed the national average,
according to the Society of Thoracic Surgeons. This not-for-profit organization’s mission is to ensure patients receive the highest quality care,

Experienced Team Approach to Care
Our team includes highly specialized pediatric and adult congenital heart experts, including cardiologists, interventional cardiologists, heart surgeons, cardiothoracic imaging specialists, anesthesiologists, and more. We combine our experience and expertise to ensure you or your child receives the best possible care.

Robust Adult Congenital Program
The Duke Adult Congenital Heart Disease Program offers advanced care to people living with congenital heart defects. Our board-certified adult congenital heart disease specialists have completed advanced training and have years of experience treating adults with congenital heart disease. We offer the full range of therapies to treat problems that arise in adults living with congenital heart disease.

Access to the Latest Treatments
From the catheterization lab to surgery and beyond, you may have access to clinical trials studying new and better ways to care for people with congenital heart defects. As a Duke patient, you may be eligible to receive new treatments before they are available elsewhere.

This page was medically reviewed on 02/12/2026 by
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