Definition

What is Eisenmenger syndrome?

Eisenmenger syndrome is a rare progressive heart condition caused by a structural error in the heart, typically a “hole in the heart” (ventricular septal defect) present at birth (congenital heart defect). This causes abnormal blood flow in the heart, resulting in high pressure within the pulmonary artery, the main blood vessel that connects the heart to the lungs (pulmonary hypertension).

How common is Eisenmenger syndrome?

Eisenmenger syndrome is uncommon. Please discuss with your doctor for further information.

Symptoms

What are the symptoms of Eisenmenger syndrome?

The common symptoms of Eisenmenger syndrome are:

  • Bluish or grayish skin color (cyanosis)
  • Large, rounded fingernails or toenails (clubbing)
  • Easily tiring and shortness of breath with activity
  • Shortness of breath while at rest
  • Chest pain or tightness
  • Skipped or racing heartbeats (palpitations)
  • Fainting (syncope)
  • Coughing up blood (hemoptysis)
  • Dizziness
  • Numbness or tingling in the fingers or toes
  • Headaches
  • Abdominal swelling

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you or your loved one has any signs or symptoms listed above or you have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes Eisenmenger syndrome?

A heart defect that causes a hole (shunt) to develop between two chambers of your heart is the most common cause of Eisenmenger syndrome. This hole causes blood to circulate abnormally in your heart and lungs. Increased blood flow returns to your lungs instead of going to the rest of your body. The blood vessels in your lung arteries become stiff and narrow, increasing the pressure in your lungs’ arteries. This permanently damages the blood vessels in your lungs.

Eisenmenger syndrome occurs when the increased pressure of the blood flow in the lung becomes so great that the direction of blood flow through the shunt reverses. Oxygen-poor (blue) blood from the right side of the heart flows into the left ventricle and is pumped to your body so you don’t receive enough oxygen to all your organs and tissues.

Risk factors

What increases my risk for Eisenmenger syndrome?

A family history of heart defects also increases the risk of a baby developing a congenital heart defect, including the possibility of developing Eisenmenger syndrome. Talk to your doctor about screening other family members for heart defects if you’ve been diagnosed with a heart defect or Eisenmenger syndrome. Please consult with your doctor for further information.

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is Eisenmenger syndrome diagnosed?

To diagnose Eisenmenger syndrome, your doctor will discuss your medical history, perform a physical examination and order appropriate diagnostic tests. These tests may include:

  • Electrocardiogram (ECG). This test records the electrical activity of the heart through electrodes attached to the skin. This test helps diagnose heart defects that cause Eisenmenger syndrome.
  • Chest X-ray. Your doctor may order a chest X-ray to look for heart and pulmonary artery enlargement.
  • Echocardiogram. During an echocardiogram (ultrasound of the heart), sound waves create detailed images of your heart. This allows doctors to see the structure of your heart and blood flow through your heart to look for heart defects.
  • Blood tests. Blood tests may be done to check your blood cell counts, which are often high in Eisenmenger syndrome. Your kidney and liver function, as well as your iron level, also may be measured with blood tests.
  • Computerized tomography (CT) scan. In this test, you’ll lie in a machine that takes images of your lungs so that your doctors can see a cross-section of them. You might also be given dye that makes the images of your lungs show up more clearly.
  • Magnetic resonance imaging (MRI). This test, which uses no X-rays, is sometimes used to get images of the blood vessels in your lungs. A computer creates tissue “slices” from data generated by a powerful magnetic field and radio waves.
  • Cardiac catheterization. In this test, doctors insert a thin, flexible tube (catheter) into an artery in your groin and guide the catheter to your heart using X-ray imaging.
    Doctors use cardiac catheterization to measure blood pressure in your blood vessels or heart’s chambers, the size of any septal defect, the pressures across the defect, and the amount of blood in your heart and lungs.
    If you need to have cardiac catheterization done, make sure you choose a cardiologist who has expertise diagnosing and treating Eisenmenger syndrome.
  • Walking test. Your doctor may order a six-minute walking test to check your tolerance to a mild level of exercise.

How is Eisenmenger syndrome treated?

Eisenmenger syndrome treatment is aimed at controlling your or your child’s symptoms and managing the condition. Although there’s no cure, medications may help you feel better, improve your quality of life and prevent serious complications.

Doctors don’t recommend surgery to repair the hole in your heart once Eisenmenger syndrome has developed, and any surgery may be life-threatening. It’s important that you’re treated by a doctor who has expertise in Eisenmenger syndrome.

Observation and monitoring

You’ll be monitored through regular visits with a congenital heart disease cardiologist. You should have an appointment with your cardiologist at least once a year. A typical evaluation generally includes a thorough review of complaints and symptoms, a physical exam, and blood tests.

Medications

Medications are the primary treatment option for Eisenmenger syndrome. You’ll need to be monitored closely by a doctor when taking medications for any changes in blood pressure, fluid volume or pulse rate.

Medications for Eisenmenger syndrome include:

  • Medications to control arrhythmias. If you have an arrhythmia, you may receive medications to control your heart rhythms.
  • Iron supplements. Your doctor may prescribe iron supplements if he or she finds your iron level is too low. Don’t start taking iron supplements without talking to your doctor first.
  • Aspirin or other blood-thinning medications. If you have had a stroke, blood clot or certain types of irregular heart rhythms, your doctor may recommend aspirin or other blood thinners such as warfarin (Coumadin, Jantoven).
    However, people who have Eisenmenger syndrome are also at increased risk of bleeding when taking these medications, so don’t take any blood thinners unless your doctor tells you to do so.
    Don’t take over-the-counter pain medications, such as ibuprofen (Advil, Motrin IB, others) or naproxen (Aleve, others), without talking to your doctor first.
  • Endothelin receptor antagonists. These medications reverse the effect of endothelin, a substance in the walls of blood vessels that causes them to narrow.
    One of these medications, bosentan (Tracleer), may improve your energy level and symptoms by lowering the resistance in your lung arteries. If you take bosentan, you’ll need monthly liver monitoring because the drug can damage your liver.
  • Sildenafil and tadalafil. Sildenafil (Revatio, Viagra) and tadalafil (Cialis, Adcirca) are sometimes used to treat high blood pressure in your pulmonary arteries caused by Eisenmenger syndrome. These drugs work by opening the blood vessels in the lungs to allow blood to flow through more easily. Side effects include upset stomach, dizziness and vision problems.
  • Depending on your condition, you may need to take antibiotics before having certain dental and medical procedures. These procedures may allow bacteria to enter your bloodstream. Antibiotics taken before these procedures can help destroy or control the harmful bacteria that may lead to an infection of your heart’s tissues (endocarditis).
    Antibiotics are recommended only before certain dental procedures (those that cut your gum tissue or part of the teeth) and procedures involving the respiratory tract, infected skin or tissue that connects muscle to bone.

Blood drawing (phlebotomy)

If your red blood cell count becomes too high and is causing symptoms such as headache, difficulty concentrating or visual disturbances, your doctor may recommend having blood drawn to help decrease your blood cell counts. Phlebotomy should not be done routinely and should only be performed after consultation with a congenital heart disease expert. You should also receive intravenous (IV) fluids when having blood drawn to help replace the lost fluids.

Heart-lung transplantation

Some people who have Eisenmenger syndrome may eventually need a heart and lung transplant or a lung transplant with repair of the hole in the heart if no other treatments prove effective.

Birth control and pregnancy

If you have Eisenmenger syndrome, becoming pregnant poses serious health risks — and can be fatal — for the mother and baby. It’s critical that women who have Eisenmenger syndrome avoid becoming pregnant.

Your doctor may recommend nonreversible birth control, such as Essure. Essure is a metal coil inserted through the vagina into the fallopian tubes that causes scar tissue to develop. This blocks the fallopian tubes.

Having your fallopian tubes tied (tubal ligation) is less often recommended due to the risks of having even minor surgery.

Birth control pills containing estrogen aren’t recommended for women who have Eisenmenger syndrome. Estrogen increases your risk of developing blood clots that could potentially block an artery to your heart, brain or lungs. Using only barrier methods, such as condoms or diaphragms, isn’t recommended due to the risk of those methods failing.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage Eisenmenger syndrome?

The following lifestyles and home remedies might help you cope with Eisenmenger syndrome:

Check with your doctor about exercise restrictions. While you shouldn’t perform strenuous exercise or sports, you may be able to do less intense physical activities. Talk to your doctor about what type of physical activity is appropriate for you.

Avoid high altitudes. Because of the low oxygen levels at high altitudes, the American College of Cardiology and the American Heart Association recommend against living at an altitude of 5,000 feet (1,524 meters) or higher above sea level. Discuss travel by airplane or to high altitudes with your cardiologist for specific recommendations.

Avoid situations that can excessively lower blood pressure. These include sitting in a hot tub or sauna or taking long hot baths or showers. These activities lower your blood pressure and cause fainting or even death. You should also avoid activities that cause prolonged straining, such as lifting heavy objects or weights.

Be cautious with any medications and supplements. Many prescription and over-the-counter medications or supplements may increase or decrease blood pressure, increase risk of bleeding or blood clots, or affect kidney function in patients who have Eisenmenger syndrome. Talk to your doctor before taking any supplements or medications.

Avoid secondhand smoke and quit using tobacco products. Cigarette smoke and other tobacco products can cause further damage to your lungs’ arteries and increase your risk of developing complications.

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

Review Date: March 30, 2018 | Last Modified: March 30, 2018

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