Tetralogy of Fallot

Tests to diagnose tetralogy of Fallot include:

• Oxygen level measurement. A small sensor placed on a finger or toe quickly checks the amount of oxygen in the blood. This is called a pulse oximetry test.
• Echocardiogram. This test uses sound waves to create pictures of the heart in motion. It shows the heart and heart valves and how well they are working.
• Electrocardiogram. Also called ECG or EKG, this test shows how the heart is beating. Sticky patches called electrodes go on the chest and sometimes the arms or legs. Wires connect the patches to a computer. The computer prints or displays results. An electrocardiogram can help diagnose an irregular heartbeat. Changes in the heart signals also may be due to an enlarged heart.
• Chest X-ray. A chest X-ray shows the shape and condition of the heart and lungs. A common sign of tetralogy of Fallot on an X-ray is a boot-shaped heart. That means the right lower chamber is too big.
• Cardiac catheterization. This test helps diagnose or treat some heart conditions. It may be done to plan surgery. The doctor inserts one or more thin, flexible tubes into a blood vessel, usually in the groin. The tubes, called catheters, are guided to the heart. During the test, doctors can do different heart tests or treatments.

Surgery used to treat tetralogy of Fallot may include:

• Temporary surgery, also called temporary repair. Some babies with tetralogy of Fallot need a surgery to improve blood flow to the lungs for the short term while waiting for open-heart surgery. This type of treatment is called palliative surgery. A surgeon places a tube called a shunt between a large artery that comes off from the aorta and the lung artery. The tube creates a new path for blood to go to the lungs. This surgery may be done if a baby is born early or if the lung arteries aren't fully developed.

  The shunt is removed during open-heart surgery to treat tetralogy of Fallot.

• Open-heart surgery, called complete repair. People with tetralogy of Fallot need open-heart surgery to completely fix the heart.

  A complete repair usually is done in the first year of life. Rarely, a person may not have surgery in childhood if tetralogy of Fallot doesn't get diagnosed or if surgery is not available. Surgery still may help these adults.

  A complete repair is done in several steps. The surgeon patches the hole between the lower heart chambers and repairs or replaces the pulmonary valve. The surgeon may remove thickened muscle below the pulmonary valve or widen the smaller lung arteries.

After complete repair, the right lower chamber doesn't need to work as hard to pump blood. As a result, the right chamber wall should go back to its usual thickness. The oxygen level in the blood goes up. Symptoms usually get better.

The long-term survival rates for people who've had tetralogy of Fallot surgery continue to improve.

People with tetralogy of Fallot need lifelong care, ideally from a healthcare team that specializes in heart diseases. The health checkups often include imaging tests to see how well the heart is working. Tests also are done to check for surgery complications.

If you have time to prepare for the medical visit, consider taking these steps.

• Make a list of your or your baby's symptoms, including any that don't seem related to tetralogy of Fallot.
• Write down your or your child's family history, including details from both sides of the family.
• Note any medicines, vitamins or other supplements taken during pregnancy if possible.
• Ask a family member or friend to come with you, if possible. Sometimes it can be hard to remember all of the information given to you during an appointment.
• Write down questions to ask the healthcare team.

For tetralogy of Fallot, some basic questions to ask your or your child's healthcare professional include:

• What's the most likely cause of this condition?
• Are there other possible causes of these symptoms?
• What kinds of tests do I or my child need? Do these tests require special preparation?
• What treatments are available, and which do you recommend?
• What are the possible complications of treatment?
• What is the outlook after surgery?
• Are there any activity restrictions?
• Can my child play sports or take part in gym class?
• Are there any brochures or other printed material that I can take home with me? What websites do you recommend?

Don't hesitate to ask other questions.

You'll usually be asked many questions, such as:

• When did you first notice your child's symptoms?
• Can you describe your child's symptoms?
• When do these symptoms occur?
• Do the symptoms come and go, or does your child always have them?
• Do the symptoms seem to be getting worse?
• Is there a family history of congenital heart defects?
• Does anything make your child's symptoms better?
• Has your child been growing and meeting developmental milestones as expected? Ask your child's healthcare professional if you're not sure.