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? 

The treatment for pulmonary atresia may depend on your baby’s specific heart anatomy. Most babies need some type of treatment shortly after birth. Your care team at Arkansas Children’s is experienced in treating pulmonary atresia and will work with you to develop the best treatment plan for your child. Treatment options may include:

• Medication to keep a connection in the heart, called the ductus arteriosus, open after birth. Usually it closes shortly after birth, but if kept open, it can help deliver blood to the lungs until the baby can have a permanent treatment.
• A catheterization procedure, called a balloon valvuloplasty. During this procedure, your child’s doctor places a thin tube (called a catheter) through a vein in the leg and into the heart. The doctor can then place another catheter with a balloon on the end of it into the valve to open it. The balloon is then deflated and removed.
• Surgery may be the only option for some babies with pulmonary atresia. In some cases, your baby may need more than one surgery.

After treatment, babies with pulmonary atresia will need to have follow-up visits with a cardiologist (a heart doctor) into adulthood. Some children may need additional treatments or surgeries. 

Length of hospital stay is variable depending on many factors, but the average is approximately two weeks.