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Eisenmenger’s Syndrome
What is Eisenmenger’s Syndrome?
Eisenmenger's syndrome is a form of congenital heart disease in which the pressure in
the pulmonary (lung) arteries is high, causing an increased resistance to blood flow in
the lungs. The syndrome can occur as a complication of ventricular septal defect, atrial
septal defect or persistent ductus arteriosus but can be associated with any congenital
disease. It is often accompanied by a shunt (a passage) of venous blood from the right
to the left side of the circulation and subsequent blueness of the face and the
extremities (i.e. fingers and toes).
Are there any symptoms?
Common symptoms of Eisenmenger’s syndrome include tiredness and
breathlessness, which may develop in late childhood or early adult life. There may also
be dizziness, chest pain, or ankle swelling, and a bluish complexion. In severe cases
heamoptysis (coughing up blood) may occur and should be treated immediately.
How is the condition diagnosed?
The diagnosis can usually be made from clinical examination and confirmed by chest xray, electrocardiogram (ECG), echocardiogram and cardiac catheterisation. For more
information on these tests please request our booklet ‘Tests for heart conditions’.
How is it treated?
Some relief may come from treatment with drugs such as ACE inhibitors and other
vasodilators. These dilate the blood vessels and help to improve the blood flow in the
heart. Controlling the increased number of red blood cells has improved the outlook in
many patients. This is done using a method similar to that used for blood donation. In
very severe cases surgical treatments may be necessary including heart-lung or lung
transplant.
Are there any risks for patients of Eisenmenger’s syndrome?
Patients with Eisenmenger’s syndrome are at increased risk of endocarditis which is an
infection of the inner lining of the heart. To help prevent this you may need to take
antibiotics before certain dental and surgical procedures. Some patients with
Eisenmenger’s are able to live normally into early adult life. Pregnancy is generally
discouraged, but you will need to discuss this with your specialist.
February, 2005