Eisenmenger (I-sun-meng-ur) syndrome is a long-term complication of an unrepaired heart problem present at birth (congenital heart defect). Eisenmenger syndrome is life-threatening.
In Eisenmenger syndrome, there is irregular blood flow in the heart and lungs.
Symptoms of Eisenmenger syndrome include:
Blue or gray skin color due to low oxygen levels (cyanosis)
Chest pain or tightness
Coughing up blood
Dizziness or fainting
Easily tiring and shortness of breath with activity
Headaches
Large, rounded fingernails or toenails (clubbing)
Numbness or tingling in fingers or toes
Shortness of breath while at rest
Skipped or racing heartbeats (palpitations)
When to see a doctor
If you have any symptoms of Eisenmenger syndrome, see your health care provider. Make an appointment even if you have never been diagnosed with a heart problem. Symptoms such as shortness of breath or chest pain require medical attention.
The goals of Eisenmenger syndrome treatment are to control symptoms, improve quality of life and prevent serious complications.
If you are diagnosed with Eisenmenger syndrome, you'll be referred to a heart specialist (cardiologist). Regular health checkups — at least once a year — are an important part of Eisenmenger syndrome treatment. It's helpful to find a cardiologist who has experience treating people who have congenital heart defects.
To diagnose Eisenmenger syndrome, your health care provider does a physical exam and asks medical history questions.
Tests to diagnose Eisenmenger syndrome may include:
Blood tests. A complete blood cell count is often done. The red blood cell count may be high in Eisenmenger syndrome. Blood tests are also done to check kidney and liver function and iron level.
Electrocardiogram (ECG). This quick and painless test measures the electrical activity of the heart. During an ECG, sensors (electrodes) are attached to the chest and sometimes to the arms or legs. Wires connect the sensors to a machine, which displays or prints results. An ECG can show how fast or slow the heart is beating.
Chest X-ray. A chest X-ray helps show the condition of the heart and lungs.
Echocardiogram. An ultrasound of the heart is called an echocardiogram. This test uses sound waves to create detailed images of the heart in motion. An echocardiogram shows blood flow through the heart.
Computerized tomography (CT) scan. A CT scan uses X-rays to create cross-sectional images of specific parts of the body. This test may be done to get more details about the lungs and lung arteries. Dye (contrast) may be given by IV for this test. The dye helps blood vessels show up more clearly on the images. CT scan images provide more-detailed information than plain X-rays do.
Magnetic resonance imaging (MRI) scan of the lungs. This test uses magnetic fields and radio waves to create detailed images of the blood vessels in the lungs.
Cardiac catheterization. A long, thin flexible tube (catheter) is inserted in a blood vessel, usually in the groin or wrist, and guided to the heart. Dye flows through the catheter to the arteries. The dye helps the arteries show up more clearly on X-ray images and video. During this test, the provider may measure blood pressure in the blood vessels and heart's chambers. Cardiac catheterization can also help show the size of any holes in the heart and blood flow in the area.
Walking test. This six-minute walking test is done to see how well you can tolerate a mild level of exercise.
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