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Eisenmenger Syndrome
Eisenmenger Syndrome most commonly occurs as the consequence of a hole in the heart, for example a ventricular septal defect (VSD) or an atrial septal defect (ASD). It can also occur due to a patent ductus arteriosus (PDA) or due to more complex heart conditions.
These conditions allow increased blood flow into the lungs, usually at high blood pressure. If the defect or hole is not closed, this will gradually cause damage to the lung arteries and as a result, the walls of the arteries gradually thicken and reduce the amount of blood that can go through the lungs. High blood pressure in the lungs is known as pulmonary arterial hypertension and Eisenmenger syndrome is one cause of pulmonary hypertension.
Eisenmenger syndrome results in less blood going through the lungs and therefore less oxygen being picked up. This results in low oxygen levels in the blood going around your body, which causes a blue tinge to lips and skin and is picked up as a low oxygen saturation on the probe that is put on your finger in the clinic.
Low oxygen levels can have consequences for many organs of the body, and can lead to problems including abnormal kidney function, gout, gallstones and strokes. Eisenmenger Syndrome is a major heart condition which cannot be cured by surgery and it needs careful follow-up in the ACHD clinic.
The boxes below describe three of the most important problems that we see in adults with Eisenmenger Syndrome
For further information about Eisenmenger Syndrome click on the links at the bottom of the page
Erythrocytosis
It means an increased number of red blood cells (erythrocytes) in your blood.
It happens as a result of having low oxygen levels. The red blood cells are the ones that carry oxygen in your bloodstream and when your body recognises that your oxygen level is low, it responds by making more red blood cells to allow more oxygen to be carried.
Erythrocytosis is actually an appropriate response of your body to low oxygen levels, but there are some downsides to having increased numbers of red cells.
It makes you more prone to conditions like gout and gallstones as well as strokes. It can also cause headaches, light-headedness and impaired alertness in some people (these are called hyperviscosity symptoms), although this is less common.
For most people it is a stable situation and it is best left alone.
For some people who get a lot of hyperviscosity symptoms, these can sometimes be improved by taking off some blood. Nevertheless this has to be monitored carefully as it can potentially cause problems itself.
Usually not, because it is the body’s appropriate response to low oxygen levels.
When you do have erythrocytosis, it is important to make sure that you drink plenty of fluid and maintain good hydration. This will make you less likely to get hyperviscosity symptoms.
Haemoptysis
Haemoptysis is the medical word for coughing up blood.
Because the blood pressure is much higher in your lungs, small lung blood vessels can rupture, causing bleeding into your lungs.
It is sometimes brought on by lung infections, like pneumonia, but it can also happen with no particular trigger.
It depends on how much bleeding occurs. Most commonly it is a small blood vessel and only a relatively small amount of blood comes out. This sort of bleeding will usually settle down by itself over a period of a few days.
If it doesn’t settle by itself, or if the amount of bleeding is large, the medical team will sometimes recommend a heart catheter to try to block off the blood vessel that is causing the bleeding.
Very large amounts of bleeding can be fatal, but this is a very uncommon situation.
If you get a chest infection it is important to see your doctor and you may need antibiotics. This will reduce the chance of the infection causing bleeding in your lungs.
If you do cough up blood you should see your GP or contact your cardiology team. They will often recommend coming into hospital for a period of bed-rest while the bleeding settles down. In most cases this will only be for a few days.
If the bleeding continues for a longer period or it is a large amount then you may need a heart catheter. This is where a very thin tube is put in through a vein in your leg and passed through your heart into the lung blood vessels. If the bleeding blood vessel can be identified, a small metal coil can be put through the catheter to block off the bleeding blood vessel.
Seeing your doctor for any chest infections and seeing them promptly if you do cough up any blood is important.
Some people who are having frequent episodes of coughing up blood can benefit from starting medicines to reduce the blood pressure in the lungs. Your medical team will talk to you about this.
Pregnancy risk
Pregnancy is very dangerous for women with Eisenmenger Syndrome. The risk of dying during pregnancy is up to 50%.
Even if you survive pregnancy, it can cause a long term deterioration of your overall health.
There are lots of changes that your heart has to deal with during pregnancy. The amount of blood going round is 40-50% more than usual, blood tends to clot more than usual, and you become mildly anaemic.
The placenta has low resistance to blood flow, meaning that it is very easy for blood to go through the blood vessels in the placenta. In women with Eisenmenger syndrome this results in more blood going around the body and less blood going through the lungs, making oxygen levels even lower than they are prior to the pregnancy.
Having a low oxygen level is also a big problem for a developing baby.
Miscarriage is much more common and if the baby is liveborn there is a high likelihood of being smaller than normal and being born premature.
Your medical team will talk to you about the options. Most doctors regard early termination of the pregnancy as the safest approach.
If you do choose to go ahead with the pregnancy you will need to have very careful follow-up with your cardiology team and your obstetric team. Often bedrest and a long hospital stay may be required.
Yes, it’s safest to avoid pregnancy if you possibly can. There are different options available, including sterilisation for you or vasectomy for your partner.
Long-term contraceptive options are also available, such as a progesterone (hormonal) rod injected under the skin or an intra-uterine device that slowly releases a very small amount of progesterone into your cervix. These can give effective contraception for up to 5 years.
Your medical team will talk to you about which option is best for you. They can also arrange for you to meet with a family planning specialist if necessary.