Living with a Fontan Circulation
Information for teenagers and adults living with a Fontan circulation
People who have a Fontan circulation were born with a heart that could not be repaired to have two pumping chambers. After a series of operations — the last one being the Fontan surgery — the blood vessels returning blood from the upper and lower body is connected straight to the lungs (pulmonary arteries), instead of passing through the right side of the heart first. Your heart then has just one pump doing all the work pushing the blood returning from the lungs all around the body.
How your blood flows now
People who have a Fontan circulation were born with a heart that could not be repaired to have two pumping chambers. After a series of operations — the last one being the Fontan surgery — the blood vessels returning blood from the upper and lower body is connected straight to the lungs (pulmonary arteries), instead of passing through the right side of the heart first. Your heart then has just one pump doing all the work pushing the blood returning from the lungs all around the body.
Diagram 1 — The Fontan circulation. Blood from the upper and lower body flows straight to the lungs, and the single ventricle pumps oxygen-rich blood out to the body.
The simplest way to picture a Fontan circulation is as a loop with four steps:
Your body uses up the oxygen in your blood.
As your organs and muscles use oxygen, the blood becomes oxygen-poor (deoxygenated).
The blood returns to your heart but skips the right side.
Blood comes back through your two large veins, the venae cavae (SVC and IVC). In a Fontan circulation these connect straight to the blood vessels going to your lungs, so it completely bypasses the right atrium and right ventricle.
The blood picks up fresh oxygen in your lungs.
In the lungs it gets a fresh supply of oxygen and releases carbon dioxide, which you breathe out. There is no pump pushing blood through your lungs — it relies on the low pressure in your veins, your normal breathing, and gravity to reach them.
Your one pump sends the oxygen-rich blood around your body.
The oxygen-rich blood returns to the left atrium and left ventricle (your one main pump), which sends it out through the aorta to your whole body — and the loop starts again.
How to stay well
Be your own advocate. Unlike paediatrics, most adult doctors are unfamiliar with the Fontan circulation and some doctors may not realise some of the special considerations needed to provide optimal care. You don’t need to know all the operations in detail but knowing the term ‘Fontan circulation’, what your usual oxygen levels are and making sure that there is a Fontan expert cardiologist involved in decision-making are some key things to help keep things on track.
Eat a healthy, well balanced diet. Being overweight can have negative effects on the Fontan circulation. A healthy diet helps to maintain an ideal weight. If obesity is a challenge there are options your doctor can offer to help you.
Keep active. Regular exercise is usually good for you and has been shown to be highly beneficial for people who have a Fontan circulation. There are differences in the best types of exercise depending on individual factors. Ask your heart team what is best for you.
Take your medicines as prescribed. Most people need a blood thinner — never stop or change them without asking your cardiology team first. There are some medicines that are not safe so check with your cardiologist what types of medications are best avoided.
Look after your teeth and gums, and tell your dentist you have a Fontan circulation before any dental work. Antibiotics are sometimes recommended before dental procedures for some people.
Avoid smoking and vaping. Ask about alcohol — with some medicines it is best avoided, and your team can guide you.
Planning a procedure or pregnancy? Make sure to chat to your cardiologist to plan ahead to ensure that you get the best possible outcome.
Contact your heart team when you notice any of these:
Swelling of your tummy, ankles, feet or face
New or worse breathlessness, or feeling unusually tired
Chest pain, a racing or very irregular heartbeat, or fainting / near-fainting
A painful, swollen or warm leg
Fever or signs of infection without an obvious cause
Feeling light-headed or dizzy, losing your appetite, or feeling very weak
Emergency — get help immediately if:
You have sudden, severe chest pain, major trouble breathing, or collapse. Call emergency services straight away and tell them you have a Fontan circulation.
Your regular check-ups
A Fontan circulation works best with lifelong specialist follow-up, with an ACHD (adult congenital heart disease) team rather than a general adult cardiologist. Regular reviews matter even when you feel well, so that any small change is picked up early. At your check-ups you may have: a heart scan (echocardiogram), an electrocardiogram (heart rhythm recording), exercise tests, blood tests, MRI or CT scans, and sometimes monitoring of your liver, lungs or kidneys. Some people also need medicines or small procedures to keep things working well.
A message to you
Living with a Fontan circulation usually means taking a little extra care every day — but most people live full, active and independent lives. Your heart team is there to help you stay well, answer your questions and plan for every stage of life.
Questions for your doctor
You may find it helpful to ask your heart team these questions:
What level of exercise and sport is right for me?
How much should I drink each day, and are there signs I should watch for?
Which medicines do I take and why — and what should I do if I forget a dose?
What do I need to know before dental work, travel or any planned procedure?
What symptoms should I contact you about, and how quickly?
ⓘ This leaflet provides general information only and is not medical advice. Always follow the advice of your cardiology team.
