Diagnosis

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

  • 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.
  • Electrocardiogram (ECG). This test records the electrical activity of the heart through electrodes attached to the skin, which can help diagnose heart defects.
  • Chest X-ray. Your doctor may order a chest X-ray to look for heart and pulmonary artery enlargement.
  • Echocardiogram. An ultrasound of the heart is called an echocardiogram. This test uses sound waves to create detailed images of your heart. An echocardiogram allows doctors to see the structure of your heart and blood flow through your heart to look for heart defects.
  • 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 produces images of the blood vessels in your lungs by using 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, and the pressures and flow across the defect. 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.

Treatment

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, because any surgery may be life-threatening. It's important that you see 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, blood tests, and additional heart-health tests.

Medications

Medications are the primary treatment for Eisenmenger syndrome. You'll need to be monitored closely by a doctor when taking medications for any changes in blood pressure, fluid levels and your pulse rate.

Medications for Eisenmenger syndrome include:

  • Medications to control irregular heart rhythms. If you have an irregular heartbeat, you may receive medications to control your heart rhythms.
  • Iron supplements. Your doctor may prescribe iron supplements if your iron level is too low. But 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 (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 sodium (Aleve, others), without talking to your doctor first.
  • Medication that relaxes blood vessel walls. Drugs called endothelin receptor antagonists are medications that 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.
  • Antibiotics. 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).

Surgeries or other procedures

If your red blood cell count becomes too high and is causing symptoms such as headache, difficulty concentrating or vision problems, your doctor may recommend having blood drawn to help decrease your blood cell counts. The blood draw procedure is called phlebotomy. It should not be done routinely and should only be done after consultation with a congenital heart disease expert. You should receive IV fluids when having blood drawn to help replace the lost fluids.

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 other treatments don't control your symptoms.

Clinical trials

Explore Mayo Clinic studies testing new treatments, interventions and tests as a means to prevent, detect, treat or manage this condition.

Lifestyle and home remedies

If you're diagnosed with Eisenmenger syndrome, you can still lead an active life with proper treatment and precautions.

  • Avoid dehydration. Ask your doctor how much fluid you need each day. You may need more fluids if you're sick, in a heated room or traveling on an airplane.
  • 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.
  • Get a flu shot. Avoiding infections is even more important for people with Eisenmenger syndrome. Experts recommend getting a flu shot every year and a pneumonia vaccination every five years.
  • Avoid secondhand smoke and quit using tobacco products. Cigarette smoke and other tobacco products can increases your risk of complications. It's important to avoid recreational drug use too.

Birth control and pregnancy

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

Effective contraceptive methods include vasectomy for the male partner, or long-acting female contraception, including an intrauterine device (IUD) or a contraceptive hormonal implant such as Nexplanon. Tying of the fallopian tubes (tubal ligation) is a very effective form of contraception, but it's less often recommended due to the risks posed by having even minor surgery.

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

Coping and support

Whether you or your child has been diagnosed with Eisenmenger syndrome, it's natural to worry, even after treatment. Although treatments can help your symptoms and improve your prognosis, you may feel stressed or nervous about your condition.

Here are a few things to keep in mind to help you cope with an Eisenmenger syndrome diagnosis and treatment:

  • Emotional difficulties. Being diagnosed with Eisenmenger syndrome is life-changing. You may need to alter your plans to have a family, and you may find yourself nervous that your condition will worsen.

    If your child has been diagnosed with Eisenmenger syndrome, he or she may feel insecure and may have emotional difficulties as he or she reaches school age. Talk to your doctor or your child's doctor about ways you can cope with these problems, which may include support groups, or a visit to a therapist or psychologist.

  • Developmental difficulties for children. Because some children who have congenital heart defects and Eisenmenger syndrome may have had a long recovery time from surgeries or procedures, they may developmentally lag behind other children their age. Some children's difficulties may last into their school years, and they may have difficulties learning to read or write, as well. Talk to your child's doctor about ways to help your child through his or her developmental difficulties.
  • Support groups. A serious medical problem for you or your child isn't easy and, depending on the severity of your condition, may be very difficult and frightening. You may find that talking with others who've been through the same situation brings you comfort and encouragement. Ask your doctor or your child's doctor if there are any local support groups.

Preparing for your appointment

If you are diagnosed with Eisenmenger syndrome, you'll be referred to a heart specialist (cardiologist). It's important to find a cardiologist who has experience treating people who have congenital heart defects.

The symptoms of Eisenmenger syndrome, such as blue or grayish skin color (cyanosis) and shortness of breath, are serious. Even if you haven't been diagnosed with a heart defect previously, these symptoms require prompt medical attention.

Because Eisenmenger syndrome is a complicated condition and because there's often a lot to discuss, it's a good idea to be prepared for your appointment. Here's some information to help you get ready for your appointment, and what to expect from your doctor.

What you can do

  • Make note of any previous heart treatments. Because Eisenmenger syndrome most often develops as a complication of a heart defect, it's important that your doctor knows about any medications you've taken or surgeries or procedures you've had if you've been previously diagnosed with a heart defect.
  • Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask whether there's anything you need to do in advance, such as fill out forms or restrict your diet. For some imaging tests, for example, you may need to fast for a period of time beforehand.
  • Write down any symptoms you're experiencing, including any that may seem unrelated to Eisenmenger syndrome. Try to recall when they began. Be specific, such as days, weeks, months, and try to avoid vague terms such as "some time ago."
  • Write down key personal information, including a family history of heart defects, pulmonary hypertension, lung disease, heart disease, stroke, high blood pressure or diabetes, and any major stresses or recent life changes.
  • Make a list of all medications, as well as any vitamins or supplements that you're taking. Also, be sure to tell your doctor if you've recently stopped taking any medications.
  • Take a family member or friend along, if possible. Sometimes it can be difficult to remember all the information provided to you during an appointment. Someone who accompanies you may remember something that you missed or forgot.
  • Write down questions to ask your doctor.

Your time with your doctor may be limited, so preparing a list of questions can help you make the most of your time together. List your questions from most important to least important in case time runs out. For Eisenmenger syndrome, some basic questions to ask your doctor include:

  • What are other possible causes for my symptoms or condition?
  • What kinds of tests will I need?
  • What treatment will I need?
  • What's an appropriate level of physical activity?
  • How often do I need to follow up with you?
  • I have other health conditions. How can I best manage them together?
  • Are there any restrictions that I need to follow?
  • Can you recommend a specialist who has experience treating Eisenmenger syndrome?
  • Are there any brochures or other printed material that I can take home with me? What websites do you recommend?

In addition to the questions you've prepared to ask your doctor, don't hesitate to ask additional questions during your appointment.

What to expect from your doctor

Your doctor is likely to ask you a number of questions. Being ready to answer them may reserve time to go over any points you want to spend more time on. Your doctor may ask the following questions:

  • Have you previously been diagnosed with a heart defect or pulmonary hypertension? If so, what treatments did you have for your condition?
  • Has one of your doctors ever said you had a heart murmur, but didn't pursue a diagnosis? If so, when was this?
  • When did you first begin experiencing symptoms?
  • Have your symptoms been continuous or occasional?
  • How severe are your symptoms?
  • What, if anything, seems to improve your symptoms?
  • Does anything make your symptoms worse?

Eisenmenger syndrome care at Mayo Clinic

Dec. 05, 2020
  1. Connolly HM. Evaluation and prognosis of Eisenmenger syndrome. https://www.uptodate.com/contents/search. Accessed Oct. 21, 2020.
  2. Connolly HM. Medical management of Eisenmenger syndrome. https://www.uptodate.com/contents/search. Accessed Oct. 21, 2020.
  3. Stout KK, et al. 2018 AHA/ACC guideline for the management of adults with congenital heart disease. Journal of the American College of Cardiology. 2019; doi:10.1016/j.jacc.2018.08.1029.
  4. Gatzoulis MA, et al., eds. Eisenmenger syndrome. In: Diagnosis and Management of Adult Congenital Heart Disease. 3rd ed. Elsevier; 2018. https://www.clinicalkey.com. Accessed Oct. 21, 2020.
  5. Ferri FF. Ventricular septal defect. In: Ferri's Clinical Advisor 2021. Elsevier; 2021. https://www.clinicalkey.com. Accessed Oct. 21, 2020.
  6. Arvanitaki A, et al. Eisenmenger syndrome: diagnosis, prognosis and clinical management. Heart. 2020; doi:10.1136/heartjnl-2020-316665.
  7. Chaix MA, et al. Eisenmenger syndrome: A multisystem disorder — Do not destabilize the balanced but fragile physiology. Canadian Journal of Cardiology. 2019; doi:10.1016/j.cjca.2019.10.002.
  8. How the heart works. National Heart, Lung, and Blood Institute. https://www.nhlbi.nih.gov/health-topics/how-heart-works. Accessed Oct. 21, 2020.
  9. Chiriac A, et al. Determinants of sudden cardiac death in adult patients with Eisenmenger syndrome. Journal of the American Heart Association. 2020; doi:10.1161/JAHA.119.014554.
  10. Connolly HM. Medical management of cyanotic congenital heart disease in adults. https://www.uptodate.com/contents/search. Accessed Oct. 21, 2020.
  11. Hjortshoj C, et al. Outcome after heart-lung or lung transplantation in patients with Eisenmenger syndrome. Heart. 2020; doi:10.1136/heartjnl-2019-315345.
  12. The impact of congenital heart disease. American Heart Association. https://www.heart.org/en/health-topics/congenital-heart-defects/the-impact-of-congenital-heart-defects#.Vi1_O36rTIU. Accessed Oct. 21, 2020.
  13. Brown AY. Allscripts EPSi. Mayo Clinic. Oct. 8, 2020.

Related

Associated Procedures

Products & Services