This leaflet aims to give you an understanding of Eisenmenger syndrome and it’s management.
Eisenmenger syndrome
Eisenmenger Syndrome occurs when a hole in the heart (or other defect) allows too much blood to reach the lungs in childhood, damaging the lung arteries and leading to high blood pressure in the lungs. This is called ‘pulmonary hypertension’.
As a result of the high pressure it is difficult for the heart to pump blood around your lungs and some blue blood that is low in oxygen leaks into the red side, often causing you to be a little blue (cyanosed).


with VSD
Symptoms
If you have lower than normal levels of oxygen in the blood this is called cyanosis and this can cause a range of symptoms but these can be very variable.
Many people feel very well with Eisenmenger syndrome for many years. Some people are short of breath on exercise and may be generally tired. Chest pains occur from time to time, but are not usually a sign of major problems. Palpitations, fainting and coughing up blood are important symptoms that you should always tell your doctor about.
Blood abnormalities
If you have cyanosis, your blood becomes thicker than normal. This can lead to headaches and loss of concentration, pains in the arms and legs and pins and needles. Some doctors also think that there is an increased risk of blood clots on the lungs and brain, but this is not proven. Sometimes thicker blood does not clot as well as it should do.
Heart pump problems (heart failure)
As the blood pressure in the lungs increases, the right side of the heart has to work harder to push the blood around the lungs.
Over time, this pumping action of the heart can become tired. This makes you more breathless on exercise and can cause fluid to collect around your ankles and stomach.
Heart rhythm problems
High pressure in the lungs causes the heart to stretch. This can lead to abnormal heart rhythms. This can give symptoms of palpitations, dizziness and fainting. Abnormal rhythms can also increase the risk of developing clots in the heart.
Treatment
There is no cure at present for Eisenmenger syndrome. Treatment is therefore aimed at controlling symptoms and preventing complications. It is tailored to each individual.
Anticoagulation
We may prescribe you blood thinning medication to decrease your chance of forming clots.
Heart medication
Water tablets (diuretics) make you pass urine more frequently and therefore reduce the amount of excess fluid in your body. This takes pressure off the heart allowing it to pump more easily. This should make you less breathless and should also decrease the swelling in your ankles and abdomen.
Medication that lower the pressures in the lungs
There are some medications that can be prescribed to try to slow the progression of high blood pressure in the lungs. These are prescribed and delivered to you by the specialist unit in Sheffield. Some of these drugs require blood tests every few months at your GP surgery.
Other points to consider
Endocarditis
All patients with congenital heart disease are at risk of infection in the heart (endocarditis). The most common way bacteria can get into the blood stream and to the heart is through the mouth so it is important to practice good dental hygiene and visit the dentist every 12 months. If you need invasive dental treatment, we recommend antibiotic cover to be taken shortly before your dental appointment. Please ask your doctor or nurse for more information. Due to the increased risk of infection we would also advise against body piercing and tattoos.
Pregnancy
Pregnancy is not recommended in patients with Eisenmenger syndrome. It carries a risk to the mother’s life of up to 50%. You should therefore use a reliable method of contraception. The combined oral contraceptive pill should be avoided but there are plenty of other progrestogen-only preparations which are very effective.
Travel/Life Insurance
Many people with Eisenmenger syndrome travel on holiday. Oxygen is not usually needed for flying but may be appropriate in some circumstances such as a long-haul flight. Please discuss this with your doctor or nurse. Remember to declare your heart condition when booking holiday insurance, to ensure you are properly covered.
Most people with congenital heart disease unfortunately cannot get life insurance.
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