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.