Tetralogy of Fallot  (TOF)

What is tetralogy of Fallot?

Tetralogy of Fallot is a complex heart condition that affects how blood moves through a baby’s heart. It’s a type of congenital heart condition, which means it’s a condition a baby is born with. 

Babies with tetralogy of Fallot have four separate heart problems:

• Ventricular septal defect (VSD): A hole in the wall separating the two lower chambers (ventricles) of the heart.
• Pulmonary stenosis: A narrowing (stenosis) of the pulmonary valve, which creates a blockage of blood to flow to the lungs.
• Overriding aorta: The aortic valve is larger than normal and sits in the wrong position, right on top of the VSD.
• Right ventricular hypertrophy: A thickening of the right ventricle of the heart.

Some babies with tetralogy of Fallot also have pulmonary atresia. This is a condition in which the pulmonary valve does not open properly.

Because of these problems with the heart’s structure, not enough blood gets to the lungs. This means babies with tetralogy of Fallot don’t get enough oxygen to the cells in their body. For this reason, most babies need surgery soon after birth.

What are the signs and symptoms of tetralogy of Fallot?

In some cases, tetralogy of Fallot is found before birth during an ultrasound or fetal echocardiogram.

When a baby is born with tetralogy of Fallot, they usually have symptoms shortly after birth. Symptoms may include:

• A blue tint to the skin (cyanosis), which may get worse when feeding or crying
• Heart murmur (a sound heard by your baby’s doctor with a stethoscope)
• Fast breathing
• Irritability
• Poor feeding
• Extreme tiredness

What causes tetralogy of Fallot?

Experts do not know what causes tetralogy of Fallot. The condition develops early in pregnancy when the heart is formed.

How is tetralogy of Fallot treated? 

Most babies with tetralogy of Fallot need heart surgery shortly after birth to patch the VSD in the lower heart chambers and enlarge the pulmonary valve or artery. The specific type of surgery your baby needs will depend on their specific heart anatomy. Sometimes, babies need several surgeries. Your care team at Arkansas Children’s is experienced in treating tetralogy of Fallot and will work with you to develop the best treatment plan for your child. After surgery, babies with tetralogy of Fallot will need to have follow-up visits with a cardiologist (a heart doctor) into adulthood. Some children may need additional treatments or surgeries.