What Is Tetralogy of Fallot?

Tetralogy of Fallot is a rare, complex congenital heart defect. A congenital heart defect is a problem with the heart’s structure that’s present at birth.

Tetralogy of Fallot involves four heart defects:

  • A large ventricular septal defect (VSD)
  • Pulmonary stenosis
  • Right ventricular hypertrophy
  • An overriding aorta

What Are the Signs and Symptoms of Tetralogy of Fallot?

  • With tetralogy of Fallot, not enough blood is able to reach the lungs to get oxygen, and oxygen-poor blood flows to the body.
  • Doctors often don’t know what causes tetralogy of Fallot. Some conditions or factors that occur during pregnancy may raise your risk of having a child who has tetralogy of Fallot. Heredity and some genetic disorders also may play a role in causing the condition.
  • Cyanosis is an important sign of tetralogy of Fallot. Cyanosis is a bluish tint to the skin, lips, and fingernails. Low oxygen levels in the blood cause cyanosis.
  • Other signs and symptoms include a heart murmur, delayed growth and development, and clubbing. Clubbing is the widening or rounding of the skin or bone around the tips of the fingers.
  • Babies who have unrepaired tetralogy of Fallot sometimes have “tet spells.” A tet spell occurs when the oxygen level in the blood suddenly drops. This causes the baby to become very blue. He or she also may have trouble breathing, become very tired and limp, not respond to a parent’s voice or touch, become very fussy, or pass out.

How Is Tetralogy of Fallot Diagnosed?

Doctors diagnose tetralogy of Fallot based on a baby’s signs and symptoms, a physical exam, and the results from tests and procedures like Echocardiography , EKG , Chest X Ray , Pulse Oximetry , Cardiac Catheterization. Signs and symptoms of the heart defect usually occur during the first weeks of life.

How Is Tetralogy of Fallot Treated?

Surgery is the only effective treatment for tetralogy of Fallot. There are two types of surgery that may be performed, including intracardiac repair or a temporary procedure that uses a shunt. Most babies and children will have intracardiac repair.

Intracardiac repair

Tetralogy of Fallot treatment for most babies involves a type of open-heart surgery called intracardiac repair. This surgery is typically performed during the first year of life. During this procedure, the surgeon places a patch over the ventricular septal defect to close the hole between the ventricles. He or she also repairs the narrowed pulmonary valve and widens the pulmonary arteries to increase blood flow to the lungs. After intracardiac repair, the oxygen level in the blood increases and your baby’s symptoms will lessen.

Temporary surgery

Occasionally babies need to undergo a temporary surgery before having intracardiac repair. If your baby was born prematurely or has pulmonary arteries that are underdeveloped (hypoplastic), doctors will create a bypass (shunt) between the aorta and pulmonary artery. This bypass increases blood flow to the lungs. When your baby is ready for intracardiac repair, the shunt is removed.