Eisenmenger Syndrome

Overview

What is Eisenmenger syndrome?

Eisenmenger syndrome develops from a certain congenital (present at birth) heart condition involving structural defects in the heart. Most people with Eisenmenger have a large hole between two chambers of the heart.

How common is Eisenmenger syndrome?

Doctors most commonly diagnose Eisenmenger syndrome in people when they are young adults. The number of people with congenital heart disease who develop the condition has decreased from 8 percent to 4 percent in the last several decades. The decrease in number of people who develop Eisenmenger Syndrome is due to ultrasound and the advancements in cardiac surgery. Eisenmenger syndrome appears to affect males and females in equal numbers.

Symptoms and Causes

What causes Eisenmenger syndrome?

Uncorrected structural defects in the heart cause Eisenmenger syndrome. Most people with this condition were born with a ventricular septal defect (hole between the two lower chambers of their heart). This hole allows more blood to flow into the lungs than is normal, causing pulmonary hypertension (increased pressure in the lung’s arteries).

After time, pulmonary hypertension damages the blood vessels in the lungs. This damage causes the blood flow to reverse direction and go back out to the body without receiving oxygen in the lungs. In order to avoid serious complications, patients may have to limit exercise and strenuous activities.

Besides ventricular septal defect, other congenital heart problems that can cause Eisenmenger syndrome include:

  • Atrial and atrioventricular septal defect – a combined hole in the upper two chambers of the heart
  • Patent ductus arteriosus – failure of the passage between the pulmonary artery (blood vessel leading to the lungs) and the aorta (the body’s main artery) to close after a baby is born
  • Truncus arteriosus – only one blood vessel leads out of the heart instead of the normal two

What are the symptoms of Eisenmenger syndrome?

Symptoms of Eisenmenger syndrome vary depending on the heart defect and affected organs. They do not usually occur until people are in their teens, 20s or 30s. The signs slowly get worse.

The most common signs and symptoms of the condition include:

Diagnosis and Tests

How is Eisenmenger syndrome diagnosed?

Doctors use several tests to diagnose Eisenmenger syndrome. In addition to a physical exam, your doctor may use:

  • Electrocardiogram. Uses small electrodes placed on the chest to measure the heart’s electric activity
  • Echocardiogram or cardiac catheterization. Can view the heart from different angles; measures pulmonary artery pressure and oxygen content
  • MRI scan. Creates cross sectional images of the structures in the heart
  • Blood tests. Measure the number of red blood cells and the amount of oxygen in the blood

Management and Treatment

How is Eisenmenger syndrome managed or treated?

Ideally, the structural defect in the heart is found early in life and surgically repaired and therefore Eisenmenger syndrome is prevented. When the heart defect is not detected and repaired before damage to the lung arteries occurs, Eisenmenger syndrome is the result. Treatment for Eisenmenger syndrome is aimed at managing its symptoms and complications.

Specialists use medications to treat pulmonary hypertension and antibiotics to prevent infections. Medications used to treat pulmonary hypertension include prostacyclin analogs (such as treprostinil [Remodulin®], epoprostenol [Flolan®]), endothelin antagonists (such as bosentan [Tracleer®]), and nitric oxide enhancers (such as sildenafil [Viagra®])

Some people receive oxygen therapy to feel more comfortable. In rare cases, if other efforts to reduce complications are not successful, doctors may treat Eisenmenger syndrome with a heart and lung transplant.

What complications are associated with Eisenmenger syndrome?

Pregnancy causes cardiovascular changes in the body that can make it life-threatening for women with Eisenmenger syndrome. Doctors advise women with the condition not to get pregnant.

In addition to its symptoms, a variety of complications can develop in people with Eisenmenger syndrome. They include:

  • Increased bleeding such as easy bruising, heavy bleeding during menstruation (a woman’s period) and coughing up blood
  • Heart failure
  • Increased risk of infections in the heart, lungs, skin and brain
  • Stroke
  • Gout (swelling in the toes and other joints)
  • Impaired kidney function
  • Bone and joint issues including scoliosis (curved spine)
  • Gallstones

Steps you can take to manage complications associated with Eisenmenger syndrome include:

  • Maintain healthy teeth and gums to avoid infection
  • Take antibiotics before dental work to prevent endocarditis (infection in the heart)
  • Get an annual flu shot

To help keep complications from arising, avoid:

Outlook / Prognosis

What is the prognosis (outlook) for people with Eisenmenger syndrome?

The outlook for people with Eisenmenger syndrome depends on the patient’s response to treatment. Many people who manage and treat their symptoms enjoy a good quality of life. Monitoring your health with regularly scheduled visits with your specialist and receiving prompt treatment for complications will help contribute to a longer, more active life.

Living With

What questions should I ask my doctor?

If you have Eisenmenger syndrome, you may want to ask your doctor:

  • How serious is this syndrome?
  • What lifestyle changes do I need to make?
  • What signs of complications should I look out for?
  • How do I know if treatment is working?

When can I go back to my regular activities?

People diagnosed with Eisenmenger syndrome often have to make lifelong lifestyle changes to manage the condition. Your doctor will tell you which activities may cause complications so you can avoid them.

Last reviewed by a Cleveland Clinic medical professional on 01/29/2019.

References

  • Adult Congenital Heart Association. Accessed 2/3/2019.Eisenmenger syndrome. (https://www.achaheart.org/your-heart/health-information/eisenmenger-syndrome/)
  • Merck Manual. Accessed 2/3/2019.Eisenmenger syndrome. (https://www.merckmanuals.com/professional/pediatrics/congenital-cardiovascular-anomalies/eisenmenger-syndrome)
  • National Organization for Rare Disorders. Accessed 2/3/2019.Eisenmenger Syndrome. (https://rarediseases.org/rare-diseases/eisenmenger-syndrome)

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