It is common for people who have a Fontan circulation to develop problems with their heart rhythm, this is called an arrhythmia. 

Fast heart beats (tachyarrhythmias) are more common than slow heart beats (bradyarrhythmias). 

What to look out for:

• shortness of breath
• feeling lightheaded
• fainting
• palpitations (feeling a fast or irregular heart beat)
• chest pain

If you notice any of these symptoms you should go to your nearest Emergency Department. 

It is possible to have an arrhythmia without experiencing the above symptoms. Regularly attending follow up appointments is important so that any problems can be prevented/detected early. 

During your appointments, your cardiologist will discuss the risk of developing liver problems. You may be asked to have a blood test to check your liver function every 1-2 years. 

You may also be asked to have a liver ultrasound, CT or MRI scan. All of these tests are non-invasive and painless. If there are any concerns seen in these tests, your cardiologist may refer you to a liver specialist.

Contraception

It is important to discuss which form of birth control is safest with your cardiologist. 

Contraceptives with oestrogen (the most common type of oral contraceptive pill) should be avoided as they can increase the risk of blood clots. 

Fertility

It is important to discuss your plans for a family with your cardiologist before you become pregnant.

Women with a Fontan circulation who become pregnant need close monitoring and planning to ensure a safe and healthy pregnancy and delivery.

There are specialised obstetricians who work with women who have congenital heart disease.

For the majority of people with congenital heart disease there is a slightly increased chance (less than 3%) that your child will be born with congenital heart disease. 

A fetal echocardiogram (ultrasound of the heart for the baby before being born) is usually recommended if one of the parents has a heart condition. 

You do not have a legal duty to tell your employer about your heart condition. However, you should inform your employer that you have a heart condition so that appropriate support can be put in to place if required.

You should also make sure your employer has correct and up to date contact details of the person you would like to be called in case of an emergency.

Adjustments can be requested like allowing you to take an extra break to take your medication. Think about what support you might need and be prepared to discuss this with your employer. 

Before getting your license, you’ll need to have a medical assessment with your GP.

If you have a pacemaker or defibrillator or have previously had a stroke, seizures or other episodes resulting in a loss of consciousness (blacking out), you will need to meet certain requirements to get a driver’s license.

This will include being free from any episodes for 6 months before obtaining your license.

Your doctor may recommend that you have a driving assessment with an occupational therapist or have modifications fitted to your car.

Your cardiologist can tell you what type of exercise and level of effort is best for you. Your cardiologist may ask you to do an exercise stress test to understand how much exercise is safe for your heart.

It may be a good idea to visit with an exercise physiologist to develop an exercise program to help you keep active. Competitive and contact sport is usually avoided. 

Having good oral health is important for the prevention of bacterial heart infections, known as endocarditis.

Many people who have a cardiac condition (including those with a Fontan circulation) have an increased risk of endocarditis due to the artificial material used during their heart surgeries.

You can reduce the risk of endocarditis by:

• regularly brushing your teeth
• flossing
• annual dentist check ups. 

You should also check with your cardiologist about whether you need antibiotics before dental procedures.