Tetralogy of Fallot

What is Tetralogy of Fallot?

Tetralogy of Fallot is a congenital heart condition that involves the incorrect formation of the septum between the right and left ventricles. This condition results in mixing of oxygen-rich and oxygen-poor blood across the ventricular septal defect inside the heart. This causes an overall decrease in the amount of oxygen in the blood.

The four functional heart problems that make up a tetralogy of Fallot are:

• A hole between the ventricles, the lower chambers of the heart. This is called a ventricular septal defect(VSD).
• A blockage or kink in the pulmonary artery where blood flows from the heart to the lungs
• The aorta, the largest blood vessel, lies over the VSD, the hole in the lower chambers of the heart
• The muscle surrounding the lower right chamber is too thick

Tetralogy of Fallot is a congenital heart defect, meaning children are born with it. The cause of the condition is not known. In some situations, it may be associated with certain genetic syndromes, like Di George syndrome.

Children usually show symptoms of the condition and are diagnosed shortly after birth. With treatment, kids with tetralogy of Fallot can lead normal, healthy lives. However, if your child has tetralogy of Fallot, he or she will need follow-up care to monitor any changes in the heart.

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How is Tetralogy of Fallot treated?

Surgery is the only definitive treatment for children with tetralogy of Fallot. Your doctors will likely schedule your child's surgery by the time he or she turns one year old.

During the surgery, a pediatric cardiac surgeon will fix the hole between the ventricles (the ventricular septal defect) using a patch. The surgeon will also widen the pulmonary artery and fix any problems with the pulmonary valve. This repair will help more blood reach the lungs. The entire procedure is known as intra-cardiac repair.

If your child is too ill or too small for intra-cardiac repair, surgeons will create a temporary solution called a shunt. This is a bypass from the aorta to the pulmonary artery, which will increase blood flow to the lungs until your child is big enough for the final procedure.

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What are the signs and symptoms of Tetralogy of Fallot?

Children with this heart condition often have a blue tint to their skin, lips and fingernails. This is called cyanosis and means that not enough oxygen-rich blood is reaching the child's body.

Sometimes, a baby only shows signs of cyanosis after crying or feeding. These episodes are called "Tet spells."

Other symptoms of tetralogy of Fallot are:

• Trouble feeding
• Poor growth and weight gain
• Fainting
• Clubbed fingers

If your child is having any of these symptoms, especially cyanosis, contact us immediately.

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Diagnosing Tetralogy of Fallot

If us suspects your child has a congenital heart defect, he or she will want to do more tests to examine the heart. These tests will help your cardiologist identify the problem affecting your child and create a treatment plan.

Tests that help cardiologists diagnose tetralogy of Fallot are:

• Chest x-ray
• EKG
• Holter and event monitors
• ECHO

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Helpful resources

If you'd like to learn more about tetralogy of Fallot, visit:

• The American Heart Association
• The National Heart, Lung and Blood Institute
• U.S. National Library of Medicine
• Tetralogy of Fallot Program at the Children's Hospital Colorado

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Reprinted with permission from Children's Hospital Colorado 2012, All rights reserved.