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Tetralogy of Fallot

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KEY POINTS

  • Tetralogy of Fallot is group of 4 birth defects in the heart. These 4 problems mean that not enough blood can get to the lungs to get oxygen. The oxygen-poor blood is pumped to the rest of the body.
  • This birth defect is usually treated with surgery in childhood.
  • Treatment may include medicines and regular follow up visits. If you have a damaged heart valve that is causing severe symptoms, you may need valve replacement surgery.
  • Make sure that you stay up to date with the recommended vaccines, such as flu and pneumococcal shots.

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What is Tetralogy of Fallot?

Tetralogy of Fallot (ToF) is group of birth defects in the heart. The heart has 4 sections, or chambers. There are two upper chambers each called atria, and the two lower chambers are called ventricles. The heart muscle squeezes to push blood through these 4 chambers to the lungs and the rest of the body. Blood flows from the right atrium into the right ventricle, and the right ventricle pumps it to the lungs. As it passes through the lungs, the blood picks up oxygen and leaves behind carbon dioxide. Then the blood flows back to the heart and into the left atrium, and from there into the left ventricle. The left ventricle pumps the blood out to the rest of the body, with a small amount going to the heart muscle itself. The heart pushes blood out through the arteries and blood returns to the heart through the veins.

The heart has 4 valves that open and close with each heartbeat to keep the blood flowing in the right direction through the heart. They are made of thin tissues that open and close easily. The valves are closed while the chambers fill with blood and then they open to push blood out.

ToF means that the heart has these 4 problems:

  • A bigger and thicker muscle in the lower right chamber of the heart
  • A hole in the wall between the 2 lower heart chambers of the heart
  • An aorta in the wrong part of the heart. The aorta is the large artery that carries blood from the heart to the rest of the body.
  • Narrowing of the valve and artery that carries blood to the lungs from the right side of the heart

These 4 problems mean that not enough blood can get to the lungs to get oxygen. The oxygen-poor blood is pumped to the rest of the body.

What is the cause?

The cause of this birth defect is not known. It may be passed from parents to children through their genes. Genes inside each cell contain the information that tells the body how to develop and work. People with heart defects have a slightly higher chance of having a baby with a heart defect. It is more common in people with Down syndrome.

What are the symptoms?

Most people with ToF have surgery to repair the defect in childhood. However, other problems with the heart may develop, such as leaky heart valves. People who don't have a lot of leaking may not have any symptoms. Over time, the added workload on your heart may cause symptoms that may include:

  • Abnormal heart rhythm that feels like your heart is pounding, racing, or skipping in your chest
  • Shortness of breath or trouble breathing
  • Feeling very tired
  • Fainting
  • Chest pain

How is it diagnosed?

Your healthcare provider will ask about your symptoms and medical history and examine you. People with this defect usually have a heart murmur, which your healthcare provider can hear with a stethoscope. A heart murmur is a whooshing sound as blood moves through the heart.

Tests may include:

  • An ECG (also called an EKG or electrocardiogram), which measures and records your heartbeat. You may have an ECG while you are resting or while you exercise on a treadmill.
  • An echocardiogram, which uses sound waves (ultrasound) to show pictures of the heart and how well blood is flowing through it
  • Heart catheterization, which uses a small tube called a catheter inserted into a blood vessel and up into the aorta. Dye is injected into a vein and X-rays are taken of the aorta and surrounding blood vessels.

How is it treated?

Most people who had surgery in childhood only need regular follow-up visits with a specialist in congenital heart disease. If you do have symptoms later in life, treatment may include medicine to:

  • Expand blood vessels and improve blood flow
  • Lower blood pressure
  • Prevent blood clots
  • Help your heart pump

If you have a damaged heart valve that is causing severe symptoms, you may need valve replacement surgery.

How can I take care of myself?

You need to have regular follow-up visits with a specialist in congenital heart disease. Regular checkups help find problems early and prevent damage to your heart. Follow the full course of treatment prescribed by your healthcare provider. In addition:

  • Take care of your health. Try to get at least 7 to 9 hours of sleep each night. If you smoke, try to quit. If you want to drink alcohol, ask your healthcare provider how much is safe for you to drink. Learn ways to manage stress. Try to keep a healthy weight. If you are overweight, lose weight. Exercise according to your healthcare provider's instructions.
  • Make sure that you are up to date with the recommended vaccines, such as flu and pneumococcal shots.

Ask your provider:

  • How and when you will get your test results
  • If there are activities you should avoid and when you can return to your normal activities
  • How to take care of yourself at home
  • What symptoms or problems you should watch for and what to do if you have them

Make sure you know when you should come back for a checkup. Keep all appointments for provider visits or tests.

Developed by RelayHealth.
Adult Advisor 2016.4 published by RelayHealth.
Last modified: 2016-03-23
Last reviewed: 2015-05-18
This content is reviewed periodically and is subject to change as new health information becomes available. The information is intended to inform and educate and is not a replacement for medical evaluation, advice, diagnosis or treatment by a healthcare professional.
Copyright © 2016 RelayHealth, a division of McKesson Technologies Inc. All rights reserved.
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