Eisenmenger complex; Eisenmenger disease; Eisenmenger reaction; Eisenmenger physiology

• 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)

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 patient'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 to the small lung arteries occurs.

Older children 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).

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

How well the infant or child 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.

• 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

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

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.