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Eisenmenger syndrome


Alternative Names:

Eisenmenger complex; Eisenmenger disease; Eisenmenger reaction; Eisenmenger physiology

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.


Review Date: 2/17/2014
Reviewed By: 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.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2002 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

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