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Tetralogy of Fallot

Tetralogy of Fallot is a relatively rare condition that affects about 3 in 10,000 live births. It refers to a combination of four congenital (present at birth) heart defects:

  • A ventricular septal defect (VSD), a hole between the heart’s right and left ventricles
  • Pulmonary stenosis, narrowing of both the pulmonary valve and the artery that connects the heart with the lungs
  • Right ventricular hypertrophy, in which the muscle of the right ventricle becomes thickened
  • An overriding aorta, in which the aorta (the main artery that carries oxygen-rich blood to the body) is not in the proper place

In babies and children with tetralogy of Fallot, the blood leaving the heart doesn’t get enough oxygen to feed the rest of the body. This can cause a variety of issues, including the following:

  • Bluing of the skin, lips and fingernails (cyanosis)
  • Breathing problems
  • Heart murmur
  • Fatigue during feedings, which can affect the baby’s ability to gain weight normally
  • Clubbed (widened and rounded) fingers

Tetralogy of Fallot: what you should know

  • These things may contribute to a woman having a baby with tetralogy of Fallot:
    • Drinking alcohol during pregnancy
    • Being over the age of 40
    • Having diabetes
    • Genetics (women who had tetralogy of Fallot or other congenital heart defects may be more likely to have children with this defect)
  • Tetralogy of Fallot and other congenital heart defects often affect children with genetic disorders such as Down syndrome, DiGeorge syndrome and others.
  • With advances in congenital heart surgery, most children with tetralogy of Fallot live to be adults. But they may need additional surgeries or medical procedures as they grow. And because their heart condition makes them vulnerable to other health problems, they require lifelong monitoring and care from cardiologists.

How can I get help for tetralogy of Fallot?

As with many congenital heart defects, signs and symptoms of tetralogy of Fallot typically appear right after or within a few weeks of birth. One of the most common telltale signs is a bluish tint to the skin, lips and fingernails — or cyanosis — due to low oxygen levels in the blood. 

You may also notice the following issues with your baby:

  • Has difficulty breathing
  • Grows very tired and limp
  • Doesn’t respond to your voice or touch
  • Acts fussy
  • Loses consciousness
  • Gets very tired during feedings (and, as a result, may not be gaining weight normally)
  • Has clubbed (widened and rounded) fingers

If your baby has any of these symptoms, call your pediatrician right away. He or she may refer you to a pediatric cardiologist in the Rush Pediatric Congenital Heart Disease Program. The specialist will do a physical exam and may run one or more of the following tests to determine whether your baby has tetralogy of Fallot:

  • Echocardiogram
  • Electrocardiogram (EKG)
  • Chest X-ray
  • Cardiac catheterization

The condition can also be diagnosed before birth with a test called fetal echocardiogram. This is done in the following instances:

  • The pregnant mother has risk factors for suspecting tetralogy of Fallot in the fetus, such as exposure to alcohol, diabetes or a family history of congenital heart defects
  • There is a suspicion that the fetus has a cardiac abnormality on obstetrical ultrasound screening

Care for tetralogy of Fallot at Rush

If your baby has tetralogy of Fallot, pediatric heart surgeons at Rush will repair all four defects with open-heart surgery to help your baby’s heart work as normally as possible.

Surgery may be done soon after birth or a few months later, depending on the following:

  • Your baby’s weight
  • Your baby’s overall health
  • The severity of the defects — especially the narrowing in the pulmonary artery
  • The severity of your baby’s symptoms

If a baby is not big or strong enough for the full repair, doctors may recommend a temporary surgery or nonsurgical procedure. This procedure will improve oxygen levels in the blood, giving the baby time to grow and get stronger. The full surgery to repair all four defects will then be performed once the baby is ready.

A pediatric cardiologist and pediatric heart surgeon will discuss your baby’s condition with you and come up with a treatment plan.

Because babies who are treated for tetralogy of Fallot are vulnerable to future heart problems, they require lifelong monitoring and care to stay as healthy as possible. Your baby’s care team will talk to you about follow-up visits and treatments — and what to expect as your child grows.

Why choose Rush for tetralogy of Fallot care

  • The Rush Pediatric Congenital Heart Disease Program provides family-centered, state-of-the-art care for infants, children, adolescents and teenagers who are born with heart defects.
  • Rush University Children's Hospital, a regional referral center, is home to compassionate experts from more than 30 specialties who care for children of all ages. At Rush, we put families first and are committed to involving you in every aspect of your child's care.

Departments and programs that treat this condition