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 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.
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).
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.
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.