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Eisenmenger syndrome

Eisenmenger syndrome is a condition that affects blood flow from the heart to the lungs in some people who were born with structural problems of the heart.

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Alternative Names

Eisenmenger complex; Eisenmenger disease; Eisenmenger reaction; Eisenmenger physiology

Causes

Eisenmenger syndrome is a condition that results from abnormal blood circulation caused by a defect in the heart. Most often, people with this condition are born with a hole between the two pumping chambers -- the left and right ventricles -- of the heart (ventricular septal defect ). The hole allows blood that has already picked up oxygen from the lungs to flow back into the lungs, instead of going out to the rest of the body.

Eisenmenger syndrome

Other heart defects that can lead to Eisenmenger syndrome include:

Over time, typically over many years, increased blood flow can damage the small blood vessels in the lungs. This causes high blood pressure in the lungs. As a result, the blood backs up and does not go to the lungs to pick up oxygen. Instead, the blood goes from the right side to the left side of the heart, allowing oxygen-poor blood to travel to the rest of the body.

Eisenmenger syndrome may begin to develop before a child reaches puberty. However, it also can develop in young adulthood, and may progress throughout young adulthood.

Symptoms

  • Bluish lips, fingers, toes, and skin (cyanosis)
  • Chest pain
  • Coughing up blood
  • Dizziness
  • Fainting
  • Feeling tired
  • Shortness of breath
  • Stroke
  • Swelling in the joints caused by too much uric acid (gout)

Exams and Tests

The doctor will examine the child. During the exam, the doctor may find:

  • Abnormal heart rhythm (arrhythmia)
  • Enlarged ends of the fingers or toes (clubbing)
  • Heart murmur (an extra sound when listening to the heart)

The doctor will diagnose Eisenmenger syndrome by looking at the person's history of heart problems. Tests may include:

  • Complete blood count (CBC)
  • Chest x-ray
  • MRI scan of the heart
  • Putting a thin tube in an artery to view the heart and blood vessels and measure pressures (cardiac catheterization)
  • Test of the electrical activity in the heart (electrocardiogram)
  • Ultrasound of the heart (echocardiogram)

The number of cases of this condition in the United States has dropped because doctors are now able to diagnose and correct the defect sooner, before the irreversible damage occurs to the small lung arteries.

Treatment

At times, people with symptoms may have blood removed from the body (phlebotomy) to reduce the number of red blood cells, and then receive fluids to replace the lost blood (volume replacement).

Affected people may receive oxygen, although it is unclear if it helps to prevent the disease from getting worse. People with very severe symptoms may need a heart-lung transplant.

Outlook (Prognosis)

How well the affected person does depends on whether another medical condition is present, and the age at which high blood pressure develops in the lungs. Patients with this condition can live 20 to 50 years.

Possible Complications

  • Bleeding (hemorrhage) in the brain
  • Congestive heart failure
  • Gout
  • Heart attack
  • Hyperviscosity (sludging of the blood because it is too thick with blood cells)
  • Infection (abscess) in the brain
  • Kidney failure
  • Poor blood flow to the brain
  • Stroke
  • Sudden death

When to Contact a Medical Professional

Call your health care provider if your child develops symptoms of Eisenmenger syndrome.

Prevention

Surgery as early as possible to correct the heart defect can prevent Eisenmenger syndrome.

References

Bernstein D. Pulmonary vascular disease (Eisenmenger syndrome). In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Saunders Elsevier; 2011:chap 427.2.

Updated: 2/17/2014

Kurt R. Schumacher, MD, Pediatric Cardiology, University of Michigan Congenital Heart Center, Ann Arbor, MI. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.


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