The normal heart has two sides. The right side delivers blue blood (low in oxygen) to the lungs, and the left side delivers red blood (high in oxygen) to the body. On each side there is a collection chamber (atrium) and a pumping chamber (ventricle).
Blue blood is returned from the body after the oxygen has been used by the body’s organs, it
arrives in the collecting chamber (right atrium) and then passes into the right sided pumping
chamber (ventricle) via a one-way valve. From here it is pumped into the lungs where it picks up oxygen and turns red. The red blood is then circulates via the left collecting chamber (atrium) the left pumping chamber through a valve. The left pumping chamber (ventricle) pumps the oxygenated red blood to the body.
In many complex cardiac conditions, there is only one functioning pumping chamber (ventricle). This means has to pump blood both to the lungs and to the rest of the body, in these cases the blue and red blood is mixed making it an inefficient circulation.
A Fontan is a series of surgical procedures that can be performed, and allows the blue blood and the red blood to be separated.
The aim of the Fontan is to increase the blood going to the lungs and reduce the workload of the single pumping chamber.
Fontan procedure is not a cure and, while very effective, it can be associated with other problems which affect the liver, gut, kidneys etc. Attending routine appointments is essential because it is
important that patients are monitored closely so that any ensuing complications can be dealt with and addressed as soon as possible. You should attend your planned appointment, even when you feel well because there could be changes you may not be aware of.
Echocardiogram (ECHO) - Looks at your heart function and how the valves are working.
Electrocardiogram (ECG)- Looks at the heart rhythm and how the heart is beating.
After the age of 14, further investigations may be performed. In the adult clinic, these tests will happen on a yearly basis.
Blood Tests - A yearly set of blood tests allow your cardiologist to assess how well the other organs in your body are coping with your Fontan circulation.
Fibroscan - This is similar to an ultra sound, to assess liver stiffness.
Ultra Sound Scan of the Abdomen - Looks at the abdominal organs.
Extra tests will be requested occasionally (every few years) to give your cardiologist more detailed information about how you heart is functioning, these include:
Cardiopulmonary Exercise Test (CPET): Is an exercise test on a bicycle, you need to wear a mask. This helps assess how well your heart and lungs are working. The test lasts about 15-20 minutes.
Exercise stress test: Connected to ECG leads, you exercise on a treadmill or a stationary bicycle so that your doctor can determine your level of condition and your heart's electrical activity, heart rate and blood pressure during exercise.
CT Scan: A test that uses x-rays and a computer to create detailed 3 dimensional pictures of the inside of your body. The whole scan takes about 15 minutes. You may require an injection to help light up the parts of the heart we need to look at.
Cardiac Catheter: This involves passing a thin tube into the heart to check the pressures and oxygen levels. A tube is inserted from the groin, neck or arm and passed along the blood vessels to the heart. It may also be necessary to take some X-ray pictures. You would normally be admitted to hospital for this.
MRI Scan: Is a scan that uses strong magnetic fields and radio waves to produce detailed images of the inside of the body. It is a large tube that contains powerful magnets. You lie inside the tube during the scan. At certain times during the scan, the scanner will make loud tapping noises. You will be given earplugs or headphones to wear. It is important to keep as still as possible during your MRI scan. The scan lasts 15 to 90 minutes. You may find it uncomfortable if you have claustrophobia, but most people are able to manage it with support from the radiographer.
Follow a heart-healthy lifestyle
There may be times when you may need a special diet because of your Fontan circulation. In these cases a dietician will be able to give you advice, accessed through the congenital heart diseases service.
Talk your consultant about whether it is safe for you to drink alcohol in moderation (no more than one drink per day for women and two drinks per day for men). Alcohol use can increase blood pressure and contribute to abnormal heart rhythms. It can also affect heart medications such as warfarin.
Regular exercise helps physical and psychological health. The majority of people born with heart problems can lead a full and active life and do not need to restrict their physical activity. Physical fitness is important in reducing the risk of both coronary heart disease and obesity. You can improve your fitness level even if your exercise capacity is reduced.
Different types of exercise produce different effects on the heart. There are basically two types of exercise:
Isotonic exercise is usually the preferred type of exercise for patients with Fontan circulation. Your consultant can advise you individually about this. People who take anticoagulants for example, are advised to avoid participation in contact sports. Some medication e.g. beta-blockers can also affect heart rate response to exercise.
Be realistic; set your own achievable goals. Aim for 30 minutes of moderate exercise such as brisk walking on at least five days a week. Physical activity is effective even when broken down into 10 minute chunks. Increase your exercise as part of daily living, activities such as walking to work and using the stairs can be as beneficial as gym based exercise.
There are many free fitness apps you can download to suit all abilities, you don’t have to spend money on going to gyms to increase your exercise tolerance or capacity:
My fitness Pal: https://www.myfitnesspal.com/
Maintaining a healthy weight is particularly important for people with congenital heart disease.
Obesity contributes to high blood pressure, heart disease in general and other diseases like diabetes. There is help available if you struggle with your weight management. Your GP is a good place to start. They may refer you to other services, such as a local weight loss groups. These could be provided by the NHS, or commercial services that you pay for.
There are many studies which have shown that doing physical activity can also improve mental health. For example:
People with congenital heart disease experience unique challenges, for example: frequent medical tests, surgical procedures; uncertainty about their condition and how it will impact on their lives; missed school or work for medical reasons; and struggles with family planning. This can lead
people to experience a range of emotions including worry, anxiety, sadness, fear and anger. All of these emotions are very normal.
However, they can be difficult for some people to manage and may impact on things that are important to them such as work, study, relationships and hobbies. Some may find it more challenging to cope than others. Anxiety or depression can make it hard to manage your cardiac condition, attend appointments and take medications.
If you feel that support from the psychology service would be helpful you can speak to one of the Clinical Nurse Specialists or your Cardiologist about a referral to Psychological Health Services.
Smoking can lead to problems like reduced circulation leading to clots in your circulation, blood vessel damage and a higher risk of stroke. Smoking can also interfere with many medications. The effects of smoking will be more pronounced in people with a Fontan circulation compared to people with a normal circulation. The Fontan circulation relies on the lungs to be in the best possible condition to maintain a good circulation. Smoking/vaping can also preclude you from transplant should this ever be required. The NHS website has more information about smoking cessation and where to seek help.
Recreational drugs should be avoided by everyone, but especially if you have a heart condition.
For advice, a GP is a good place to start. They can discuss your problems with you and get you into treatment. They may offer you treatment at the practice or refer you to your local drug service.
If you're not comfortable talking to a GP, you can approach your local drug treatment service yourself.
Visit the Frank website to find local drug treatment services.
Endocarditis is an infection of the inner wall of the heart and /or the heart valves caused by bacteria that enter the blood stream. It is serious and requires action. It is not a common infection but people with a heart conditions are at a greater risk of developing it than those with a normal heart. You can get endocarditis more than once.
The following factors can increase the risk of endocarditis:
Due to your heart condition it is important that you maintain good oral health by eating a healthy diet, avoiding frequent sugary foods or drinks, brushing twice daily with a fluoride toothpaste, and attending a dentist regularly. By looking after your mouth and teeth it reduces your chance of having Infective
Endocarditis (a rare infection of the lining of your heart with bacteria commonly found in mouths), or needing dental care that may be more difficult to do due to your heart condition. It is important that you mention your heart condition to your dentist as you may need antibiotics before treatment (to prevent infection) and may be at increased risk of bleeding following certain dental procedures. Your dentist will be able to advise you, or can get advice from colleagues.
Should you have any concerns or queries please contact your dentist in the first instance or the ACHD Nurse Specialists.
Women with a Fontan circulation have a high rate of miscarriage, and a high rate of obstetric and neonatal complications including pre term delivery, small babies and post-partum haemorrhage. It is generally not recommended, however in some cases it is possible. Consideration of all the options can be discussed with your cardiologist and obstetrician to plan if pregnancy is safe for you and your baby. It is important to avoid unplanned pregnancies.
It is also important that you know what types of contraception are okay for you to use as not all of them are suitable for people with congenital heart disease. Patients with the Fontan circulation should not have oestrogen and should take progesterone only medication. Our aim is to give you as much information as possible so that you can make choices about your future when you are ready.
Many of the patients with a Fontan circulation are on anticoagulation medication, which can cause heavy periods. Your consultant can advise you how to manage these. There are medications that can be prescribed to help.
We provide have a joint monthly obstetric clinic for patients. It is provided jointly with the maternity team from the Royal Victoria Infirmary Hospital. If you are pregnant or planning a pregnancy inform us as soon as you can and you will be given an appointment for this clinic.
You will need to seek advice from your cardiologist or specialist nurse to check that you able to fly or under take a long journey. You may need to take a completed ‘fit to travel’ form or a certificate from your doctor. In some cases the Travel agents will arrange for this to be completed. Think carefully about your holiday destination, you will want to be able to seek medical help as quickly as possible in the event of an emergency.
For advice ask at your outpatient appointment or contact the Adult Congenital Nurse Specialist.
The most important point to remember is to ensure that you declare your heart condition when booking travel insurance. Any omissions or mistakes made in relation to your medical condition can be used as grounds to refuse your claim if made.
The following groups advise about travel in their websites:
The Somerville Foundation www.thesf.org.uk
Little Hearts Matter https://www.lhm.org.uk/information/lifestyle-information/travel-and-trips/
Pumping Marvellous https://pumpingmarvellous.org/
When a long flight is at high altitude there is less oxygen in the aircraft cabin making it harder for someone with low oxygen saturations to keep their oxygen levels high enough. For this reason your cardiologist may advise travelling with oxygen. In some cases the doctor will suggest that oxygen is given throughout the high altitude part of the flight.
If oxygen is prescribed you MUST discuss this with the airline you are going to travel with before you book.
You may also require a flight assessment test prior to your journey which your consultant can arrange.
Having a Fontan does not prevent you from driving; however heart conditions that may cause sudden attacks of disabling giddiness or fainting can. These include if you have had a pacemaker or a cardioverter defibrillator (ICD) implanted. You must tell the DVLA and stop driving.
Your cardiologist will tell you when you can drive again by consulting the relevant medical fitness standards published on GOV.UK. If you have any doubts about your ability to drive safely, speak to your doctor. They can advise you whether you should tell DVLA about your condition.
Further information can be found on the DVLA website
It is important that you know where to get the right career advice as you need to be realistic about your career options. Those with rigorous fitness tests such as the armed forces or police may not be an option. However that is not to say that there is not the right job out there for you.
You may feel that your heart condition may make it difficult for you to find a job.
The website https://nationalcareers.service.gov.uk/ is a useful resource with information about disability employment advisers who can help you to find a job to suit you or help in practical ways to enable you to work.
Having a Fontan circulation does not need to be a barrier if you want to go to consider going college or university. There is an increasing awareness of the needs of students with medical conditions.
Support can be provide in a number of ways and you may be able to get extra financial help. The following websites have lots of useful information for you to consider, from application to attending further education.
More information on benefits and allowances can be found on the following websites.
It can be difficult for some people who have congenital heart disease to get insurance. This is often because companies do not have the medical knowledge relating to specific congenital heart conditions. This can cause problems when trying to buy a house or get life insurance.
The best advice is to shop around. It is important that you inform the insurance company of your medical condition to ensure that you are fully covered. There are some more sympathetic insurers who can be contacted through the Somerville Foundation or Pumping Marvellous