Eisenmenger Syndrome

What is Eisenmenger syndrome?

Eisenmenger syndrome is an advanced form of pulmonary artery hypertension. In this condition, the arteries that carry blood from the heart to the lungs narrow. This makes the pressure of blood flow against the walls of the arteries (blood pressure) too high. The heart must work harder to pump blood into the lungs. This causes lung damage. 

Eisenmenger syndrome mostly affects teens and adults with certain heart defects. It can also affect children, although it is not as common. When the heart defects are not repaired or repaired later in life, it increases the risk for this condition. In some people, it may also occur even when the defect is repaired. 

What causes Eisenmenger syndrome?

Eisenmenger syndrome develops over time. It is a result of high blood pressure in the lungs related to certain heart defects present at birth (congenital). Defects more likely to cause this are those where the blood flows from the left side of the heart to the right side of the heart (left-to-right shunt).

Eisenmenger syndrome is more likely in larger heart defects.

What are the symptoms of Eisenmenger syndrome?

The following are the most common symptoms of Eisenmenger syndrome:

  • Blue or grayish skin from less oxygen in the blood (cyanosis)
  • Shortness of breath with activity
  • Shortness of breath at rest
  • Feeling tired
  • Chest pain or chest tightness
  • Skipping or racing heart beats
  • Headache
  • Dizziness or fainting (syncope)
  • Numbness or tingling of fingers and toes, or both
  • Blurred vision
  • Changes in the fingers and toes (clubbing)
  • Swelling
  • Enlarged liver

How is Eisenmenger syndrome diagnosed?

Your child's healthcare provider will ask about your child’s symptoms. The provider may see signs when he or she examines your child. Your provider may hear an abnormal heart sound when listening to your child's heart with a stethoscope.

Your child may be referred to a pediatric cardiologist. This is a doctor with special training to treat heart problems in babies and children.

Your child may need tests. These may include:

  • Pulse oximetry. A small probe is used to check the amount of oxygen in the blood.
  • Blood tests. Tests may be done to check for a high or low red blood cell counts.
  • Chest X-ray.  A chest X-ray shows the heart and lungs. There may be changes in the lungs because of extra blood flow.
  • Electrocardiogram (ECG).  An ECG records the electrical activity of the heart. It also shows abnormal rhythms, and detects heart muscle stress.
  • Echocardiogram (echo). An echo uses sound waves to make a moving picture of the heart and heart valves. An echo can show the direction of blood flow. It can find out the size of a defect. Different types of echoes may be done.
  • Pulmonary (lung) function testing. Lung function testing checks how well your child's lungs work.
  • Cardiac catheterization. A cardiac cath gives very detailed information about the structures inside the heart. The doctor puts a small flexible tube (catheter) into a blood vessel in the groin. The doctor guides the catheter to the heart. He or she measures blood pressure and oxygen. In some cases, the doctor may inject contrast dye to more clearly see the blood flow and structures inside the heart. 
  • Cardiovascular magnetic resonance imaging (CMRI). This test makes detailed images of the heart and blood vessels. MRI may be used to check the amount and direction of blood shunting.
  • CT scan and CT angiography. CT may be used to check for blood clots in the lung arteries and lung.
  • Pulmonary function testing. This is used to check how well the lungs work.
  • Stress test. This is an ECG done while the child is exercising.

How is Eisenmenger syndrome treated?

Treatment will depend on your child’s symptoms, age, and general health. It will also depend on how severe the condition is.

Treatment focuses on lowering the pressure in the pulmonary artery. It also aims to bring more oxygen to the lung tissues and ease the cyanosis.

Medical treatment

Medical treatment helps manage symptoms. Treatments include:

  • Medicine. Your child will be given medicines that dilate blood vessels and lower blood pressure in the lungs.
  • Oxygen. Your child will get oxygen while sleeping or resting, or continuously.
  • Phlebotomy. The doctor may remove some blood if your child has a high red blood cell count and thickened blood.

Other treatments

  • Advanced therapies. Other medicines are available that dilate or relax the arteries in the lungs.
  • Lung transplant or heart-lung transplant may be considered.

What are the complications of Eisenmenger syndrome?

Complications of this condition may include:

  • Blood clots. These may be in the deep veins in the legs (deep vein thrombosis)
  • Bleeding (hemorrhage)
  • Stroke
  • Infected material in the brain (abscess)
  • Gout
  • Irregular heart rhythms (arrhythmias)
  • Heart failure
  • Sudden death

Living with Eisenmenger syndrome

Some children with Eisenmenger syndrome may live into middle adulthood. A few may live into their 50s or 60s.

Recommendations for children with Eisenmenger syndrome include:

  • Anesthesia and surgery are considered high risk and should be carefully planned. If your child needs surgery, the surgeon and other healthcare providers should work with your child's pediatric cardiologist.
  • Your child should avoid high altitudes. Your child may be able to fly as long as the plane is pressurized. Using oxygen during flights may also decrease the chance of complications.
  • Your child should not smoke or drink.
  • Coughing should be controlled or prevented with cough-suppressing medicines. This helps lower the risk of bleeding from the lungs.
  • Make sure your child has a flu shot every year. Talk with your child's healthcare provider about the pneumococcal vaccine and other important vaccines.

Talk with your child's healthcare provider about the specific long-term outlook for your child, teen, or young adult with Eisenmenger syndrome.

When should I call my child's healthcare provider?

Worsening of symptoms or new symptoms can be early warning signs. Call your child's healthcare provider as soon as possible.

Call 911 or your local emergency service if your child has any severe symptoms. These include:

  • Serious bleeding
  • Severe headache
  • Fainting
  • Loss of consciousness

Key points about Eisenmenger syndrome

  • Eisenmenger syndrome means that the arteries that carry blood from the heart to the lungs are narrowed. This puts too much pressure on the walls of the arteries and damages the lungs. 
  • The condition mostly affects teens and young adults with certain congenital heart defects that are not repaired or repaired late.
  • Treatment for Eisenmenger syndrome aims to lower the pressure in the pulmonary artery, bring more oxygen to lung tissues, and decrease cyanosis. 
  • It is important to work with your child's healthcare team to reduce the risk of complications.

Next steps

Tips to help you get the most from a visit to your child’s healthcare provider:

  • Know the reason for the visit and what you want to happen.
  • Before your visit, write down questions you want answered.
  • At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.
  • Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.
  • Ask if your child’s condition can be treated in other ways.
  • Know why a test or procedure is recommended and what the results could mean.
  • Know what to expect if your child does not take the medicine or have the test or procedure.
  • If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
  • Know how you can contact your child’s provider after office hours. This is important if your child becomes ill and you have questions or need advice.