Tetralogy of Fallot

Tetralogy of Fallot explained

Tetralogy of Fallot (also known as Fallot's tetralogy), is a type of congenital heart defect that affects how your heart works and how blood flows through it. Tetralogy of Fallot is made up of four different problems. They are:

Pulmonary stenosis

This means that the pulmonary valve (which lets blood flow from your heart to your lungs) is narrowed. The narrowing stops your blood from flowing as easily as it should, so your heart needs to work harder to pump blood through your body.

Right ventricular hypertrophy

This is when your right ventricle (one of your heart’s main pumping chambers) grows larger than it should, and the muscle becomes thicker. Your right ventricle gets too large because it’s working harder to pump blood through your narrowed pulmonary valve.

Ventricular septal defect (VSD)

VSD is a hole in the wall between the main pumping chambers of your heart (the left and right ventricles). When your heart beats, blood flows through the hole instead of around your body.

Over-riding aorta

An over-riding aorta is when your aorta grows in the wrong place and sits above the VSD. Blood with a lack of oxygen in it is sent around your body which can cause your skin and lips to turn blue or grey (cyanosis).

Tetralogy of Fallot is a type of congenital heart disease, which means it’s something you’re born with. Many people with tetralogy of Fallot live a normal life, but over time it can cause other problems with your heart like heart failure and high blood pressure.

What is cyanosis?

Cyanosis is when your skin and lips turn blue or grey due to low levels of oxygen. It can be a side effect or symptom of some congenital heart diseases. It happens with tetralogy of Fallot when:

• blood with lots of oxygen and blood with little oxygen mixes
• this mixing of blood is caused by the VSD and the over-riding aorta
• this can lead to lower oxygen levels which can cause problems in other parts of your body.

Symptoms of tetralogy of Fallot

The symptoms of tetralogy of Fallot are different for adults and children. Symptoms for children may include:

• blue or grey skin (cyanosis)
• fatigue (getting tired easily) when playing
• shortness of breath when feeding or playing
• long periods of crying as a baby
• difficulty feeding or gaining weight
• a heart murmur.

Symptoms for adults may include:

• blue or grey skin (cyanosis) if untreated
• shortness of breath, especially when being active
• fatigue (getting tired easily) when being active
• fainting
• light-headedness
• palpitations (feeling your heartbeat pounding or beating more quickly than usual)
• a heart murmur.

Diagnosing tetralogy of Fallot

Most adults with tetralogy of Fallot have been diagnosed in childhood and know they have this condition. It’s sometimes diagnosed before you’re born during an ultrasound (a test that creates detailed images of a baby during pregnancy).

If your doctor thinks you may have tetralogy of Fallot, they’ll send you for tests to find out. The tests may include:

• chest X-ray
• echocardiogram
• electrocardiogram (also called ECG or EKG)
• oxygen level measurement.

Your doctor may ask you to have a certain type of heart catheterisation to assess your heart in more detail. Speak to your doctor if you have questions about this test, other tests or diagnosis.

Learn more about the risk factors for congenital heart disease.

Heart murmur

Some people with tetralogy of Fallot have an extra noise when their heart beats. This is called a heart murmur and it’s caused by your blood not flowing smoothly through your heart. Your doctor may hear this when they listen to your chest and send you for an echocardiogram to check if everything’s okay with your heart.

Treating tetralogy of Fallot

Surgery is the only way to treat tetralogy of Fallot. This is usually done in childhood. You may need or have had more than one operation to repair the problems with your heart. If you’re worried, or have questions about what procedures you’ve had, speak to your healthcare team.

Shunt operation

A shunt operation (or temporary operation) is one of the first procedures you may have had if you were born with tetralogy of Fallot. It’s a surgery that improves the blood flow to your lungs while you wait for complete repair surgery. It’s not always needed, but if it is, it’s done within the first four to six months of your life.

For more information on shunt operations, talk to your healthcare professional.

Complete repair

Open heart surgery is needed to repair tetralogy of Fallot. The surgery is often done within the first few years of your life. Complete repair surgery usually includes:

• a patch being sewn over the hole in your heart
• the widening of your narrowed pulmonary valve and artery
• part of your thickened pulmonary valve may also be removed.

You’ll usually stay in hospital for a few weeks after complete repair surgery. You’ll be prescribed medicines to take when you get home from hospital for a short time.

Find more information and support on congenital heart disease in children.

Surgery in adulthood

As you get older, you may have more problems with your heart that need to be treated.

A common problem for people with repaired tetralogy of Fallot is a leaky heart valve between the heart and lungs. This is known as pulmonary regurgitation, where your pulmonary valve does not close properly. This lets blood leak back into your heart instead of forwards into your lungs.

A leaky heart valve puts extra strain on your heart, making it work harder. This can cause symptoms like feeling more tired and out of breath. Over time, it can increase your risk of having a heart attack, stroke and other heart conditions.

Learn more about the symptoms, diagnosis and treatment of a leaky heart valve.

If you have repaired tetralogy of Fallot, you may be at risk of having other issues like:

• abnormal heart rhythms
• blood leakage through your VSD
• endocarditis
• heart failure.

If you're worried about surgery, read our tips to help you feel better.

Living with tetralogy of Fallot

After it’s been repaired, most people living with tetralogy of Fallot live a normal life. You’ll be offered appointments at a congenital heart disease specialist clinic, where they check your heart for the rest of your life. Even if you feel well, going to these appointments is important because there may be changes in your heart before you get symptoms.

There are a few other things to keep in mind when living with tetralogy of Fallot, like:

Family planning

If you’re pregnant or planning to have a family, it's important to let your doctor know so you can get support and specialist care. There may be more risk of problems if you have tetralogy of Fallot.

Endocarditis

This is a rare infection that causes inflammation of the valves and inner lining of the heart. People with congenital heart conditions, including tetralogy of Fallot, have an increased risk of endocarditis. To reduce the risk of developing endocarditis, your doctor or dentist might recommend antibiotics before or after any surgeries or procedures.

Staying active

If your tetralogy of Fallot has been repaired and you do not have an obstruction or leak, you may be able to enjoy exercise fully. If you still have some complications after surgery like a leak, you may not be able to do some activities. It’s best to talk to your doctor or cardiology team about the activity you’d like to do.

Emotional support

Living with a congenital heart disease like tetralogy of Fallot can be uncertain and emotionally challenging for you and your loved ones. Learn about different ways of coping from people who know.

Heart Helpline and more support

If you have any questions or concerns, speak to your healthcare team. You can talk to them about anything, even if it feels uncomfortable or embarrassing. Most people feel much better once they do.

Here are some other ways you can get support:

• Learn more about congenital heart disease.
• Speak to our cardiac nurses on Heart Helpline by phone, callback, email or online chat (Monday to Friday, 9am to 5pm).
• Contact us to talk to our customer care advisors, find your local BHF shop and for any comments, compliments and complaints you may have.
• Talk to others with heart conditions by joining a support group or online community.