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Pediatric Tetralogy of Fallot (TOF)

What is Pediatric Tetralogy of Fallot (TOF)?

Tetralogy of Fallot (TOF) is a congenital (present at birth) heart defect that occurs when children are born with a group of four (tetralogy) related heart defects. These defects keep oxygen-poor blood from reaching the lungs where it would normally become oxygen-rich. As a result, oxygen-poor blood leaves the heart and flows through your child’s body.

What are the signs and symptoms of Pediatric Tetralogy of Fallot (TOF)?

Tetralogy of Fallot (TOF) is a heart defect that occurs when a child is born with four different heart problems.

  • Ventricular septal defect (VSD) - A hole between the left and right chambers of the heart.
  • Pulmonary stenosis - Narrowing of the main blood vessel that carries blood from the heart to the lungs.
  • Ventricular hypertrophy - Thickening of the right ventricle in the heart chamber.
  • Overriding aorta - Instead of laying over the ventricular septal defect, your child’s aorta is out of place preventing it from carrying oxygen-rich blood to the body. 

In some cases, you may know your baby has Tetralogy of Fallot (TOF) before he or she is born. Some symptoms may show up during specialized testing offered through our fetal heart program. However, many children with tetralogy of Fallot (TOF) develop symptoms shortly after birth.

These symptoms include:

  • Skin that appears ashen or blue from poor circulation (cyanosis). In children with tetralogy of Fallot, this symptom is more severe and affects the lips, nail beds, arms and legs. These symptoms get worse when your child is anxious, cries or has a bowel movement (“tet spells”)
  • Difficulty gaining weight
  • Heart murmur
  • Difficulty feeding
  • Poor development
  • Hunching body over the knees or squatting down in older children

How is Pediatric Tetralogy of Fallot (TOF) diagnosed?

Accurately diagnosing Tetralogy of Fallot (TOF) helps us make sure your child gets the treatment he or she needs as soon as possible – even on their first day of life. We start with a comprehensive exam, which may include one or more separate tests.

The physical evaluation may include:

  • Observing your child’s general appearance including their size
  • Looking for physical signs of a heart defect, such as bluish skin (cyanosis) and rapid breathing
  • Listening to your child’s heart
  • Looking for signs of related conditions such as DiGeorge syndrome
  • Questions about family history, specifically the mother’s health during pregnancy

Diagnostic testing may include:

  • Electrocardiogram
  • Echocardiogram
  • Chest X-ray
  • Cardiac catheterization
  • Heart MRI
  • Pulse oximetry
  • Genetic testing

With years of experience and specialized equipment for our littlest patients, we specialize in fast and accurate testing. Learn more about the full range of tests we offer in our cardiac imaging department.

What are the causes of Pediatric Tetralogy of Fallot (TOF)?

Tetralogy of Fallot (TOF) develops before birth. While there is no known cause, a child is at a higher risk for this condition if their mother experiences certain health problems during pregnancy.

These problems include:

  • Alcohol abuse
  • Poor nutrition
  • Certain viral illnesses such as rubella
  • Diabetes
  • Pregnancy at age 40 or older (advanced maternal age)

How is Pediatric Tetralogy of Fallot (TOF) treated?

At Children’s Health, we deliver compassionate care that meets your child’s every need. From the moment your child is born, we are ready to deliver life-saving treatments.

In severe cases, a child’s oxygen levels become dangerously low, requiring immediate treatment with a medication called prostaglandin. Prostaglandin provides temporary relief by helping blood flow to the lungs until your child can have corrective surgery.

Long-term treatment for tetralogy of Fallot includes surgical repair, also known as intracardiac repair. Because children with tetralogy of Fallot are not getting enough blood flow and are at risk for life-threatening complications, we often perform surgery within the first year of life.

Intracardiac repair includes:

  • Widening the narrowed pulmonary artery
  • Widening or replacing the pulmonary valve
  • Enlarging the passage from the right ventricle to the pulmonary artery
  • Closing the ventricular septal defect with a patch

These procedures improve blood flow to the lungs and allow blood to get enough oxygen to meet the body’s needs. As a result, your child’s symptoms should go away right after surgery.

As one of the most active heart centers in the country, Children’s Health performs hundreds of heart surgeries every year. Learn more about our cardiothoracic surgery program. 

Pediatric Tetralogy of Fallot (TOF) Doctors and Providers

Frequently Asked Questions

  • Can tetralogy of Fallot (FOA) be cured?

    Yes. Surgical repair of heart defects can increase blood flow to your child’s lungs and increase oxygen levels in the blood, relieving all symptoms.

  • Once the surgical repair is complete, will my child need any additional surgeries?

    Possibly. Your child’s pulmonary valve could narrow again or not close as completely over time. If this happens, he or she may need an additional surgery in their teens to widen or replace it.

  • Will my child be able to live a normal life with Tetralogy of Fallot (FOH)?

    Corrective surgery will greatly improve your child’s condition allowing him or her to have a very normal life.