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Living With Congenital Heart Defects by Ben's Friends

ASD/Septal Defects - Aortic and Vessel Defects - Single Ventricle Defects - Tetrology of Fallot - Transposition of the Great Arteries - Valve Defects

Living With Congenital Heart Defects

Tetralogy of Fallot - as an adult

Tetralogy of Fallot (ToF) is a complex heart defect. If you have ToF, you are born with four different heart problems:

  • A hole in the wall between your heart’s main pumping chambers (ventricular septal defect or VSD)
  • A valve between your heart and lungs that is too narrow (pulmonary stenosis or PS)
  • A right heart chamber with walls that are too thick (right ventricular hypertrophy)
  • A major blood vessel (aorta) that is misplaced or moved (overriding aorta)

These problems cause the blood entering and leaving your heart to mix. Blood that is low in oxygen (“blue”) mixes with blood that is oxygen-rich (“red”). Babies born with ToF sometimes are called “blue babies” because the lack of oxygen can makes them look blue (cyanotic).

image
LEGEND

RA - Right Atrium
RV - Right Ventricle
LA - Left Atrium
LV - Left Ventricle
SVC - Superior Vena Cava
IVC - Inferior Vena Cava
MPA - Main Pulmonary Artery
Ao - Aorta
AoV - Aortic Valve
TV - Tricuspid Valve
MV - Mitral Valve
PV - Pulmonary Valve

Image Courtesy of the Centers for Disease Control and Prevention, National Center on Birth Defects and Developmental Disabilities

If I had my ToF repaired as a child am I at risk for new heart problems?

Most adults with ToF had a “complete ToF repair” in childhood. In this repair, surgeons close the hole in the heart and open up the narrow heart valve to the lungs (the pulmonary valve). While most people who undergo this repair feel well and have few problems in childhood, this does not mean that their hearts are fixed permanently. As people born with ToF age, new heart problems can develop. As adults, it’s important that they stay in the care of an adult congenital heart specialist.

What is the most common heart problem in adults with repaired ToF?

The most common problem for adults with ToF is a leaky valve between the heart and lungs (pulmonary regurgitation or PR). As part of the ToF repair, surgeons usually stretch out the valve between the heart and lungs (pulmonary valve). This almost always makes the valve leak. As people age, this leaking can get worse.

How would I know if I have a problem with a leaky valve?

A leaky valve can be a sneaky valve! There are often few or no symptoms to alert you. The first sign can often be rhythm problems, like an unusually fast heartbeat or feeling your heart “flutter.” If your valve has been leaking a lot and for a long time, you may feel tired. It is important to see your ACHD cardiologist regularly to know how and when to treat a leaky valve.

What other health problems might I be at risk for?

If you are an adult with repaired ToF, you may be at risk for an enlarged aorta. This can cause your aortic valve to leak and require an aortic root replacement. This is a surgery to replace the stretched-out part of your aorta and/or aortic valve. Less than 10% of people with ToF may have a leaking tricuspid valve, the valve between your right atrium (receiving chamber) and your right ventricle (pumping chamber). This is known as tricuspid regurgitation or TR and can only be repaired through surgery.

Other possible health problems include heartbeats that are too fast or too slow (you can learn more in the educational sheet, “The Heart and Its Electrical System,” on the ACHA website). You may also have leaking around the area that closed the ventricular septal defect (VSD), and you may be at risk for lung problems or problems with the heart muscle. Talk to your ACHD heart doctor about what you need to look for.

It is important to see your cardiologist regularly so that any changes in your heart can be detected early. Early detection can help prevent permanent damage.

Do I need special care for my ToF as an adult?

Yes. The American Heart Association and the American College of Cardiology classify ToF as moderately complex heart disease. This means that you should get your care at a special center and see your cardiologist regularly, at least every two years. Regular check-ups can help detect any problems early on and prevent long-term damage to the heart.