Eisenmenger's Syndrome

Treatment

Treatment for people with Eisenmenger's syndrome is aimed at controlling symptoms and preventing complications.

Observation and monitoring

Observation and monitoring require regular evaluation by an adult congenital cardiologist. A typical evaluation would generally include a thorough physical exam, electrocardiogram, chest X-ray and an echocardiogram. A Holter monitor or event recorder may also be used to check for irregular heart rhythms.

A therapeutic phlebotomy (removal of some blood) should not be done routinely. If therapeutic phlebotomy is used, it is usually not required more than three times a year. More frequent phlebotomies can deplete the body of iron and cause iron deficiency anemia.

Noncardiac surgery — Even a simple operation requiring a general anesthetic (such as an appendectomy) is potentially life threatening for a patient with Eisenmenger's syndrome. Surgery should be arranged with the congenital cardiologist, the surgeon and cardiac anesthesiologists and should be performed at a medical center where the physicians have special expertise in dealing with congenital cardiac problems and Eisenmenger's syndrome.

Medication

Medication is the primary treatment option for patients with Eisenmenger's syndrome. Medication is used to control rhythm disturbances, optimize heart-muscle contraction, and treat the symptoms of heart failure such as fluid buildup in the legs, abdomen and lungs. Occasionally, medication is used to help dilate the arteries in the lungs.

Many medications used to treat the symptoms of Eisenmenger's syndrome will affect blood pressure, fluid and electrolyte (sodium, potassium, etc.) values and heart rate. These medications require close monitoring. A rapid change in blood pressure, fluid volume or pulse rate could cause more right-to-left shunting and decrease oxygen levels to a critical level. Any medication that could cause blood pressure to fall should be carefully reviewed with an adult congenital cardiologist.

• Pulmonary vasodilator medications have recently been identified as an effective medical therapy for some patients with Eisenmenger's syndrome and may help to reduce symptoms related to severe pulmonary hypertension.
  
  
• Anticoagulants (blood thinners) such as aspirin or Coumadin are rarely used. If a doctor elects to prescribe anticoagulants, the patient should be monitored closely.
  
  
• Iron replacement should only be done if the blood iron level is low. Daily use of iron is not routinely recommended. Over-the-counter multivitamins will often have iron added to them. Talk to your doctor before taking a daily vitamin supplement.

Cardiac surgery

Surgery to repair the defect once Eisenmenger's syndrome has developed is not possible and may be detrimental. The resistance to forward blood flow through the lung and the increased pressure needed to push blood through the lung would cause the repaired heart to enlarge and fail.

Heart and lung transplantation or lung transplant with repair of the shunt defect are options for some people with Eisenmenger's syndrome. Heart and lung transplantation is a very serious undertaking and fewer than 50 are performed in the United States every year. As donor availability, surgical technology and drug research continue to advance, long-term results will also improve.