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Eisenmenger syndrome is a condition that affects blood flow from the heart to the lungs in some babies who have structural problems of the heart.

Eisenmenger syndrome is caused by a defect in the heart. Most often, babies 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.

Other heart defects that can lead to Eisenmenger syndrome include:

• Atrioventricular canal defect
• Atrial septal defect
• Cyanotic heart disease
• Patent ductus arteriosus
• Persistent truncus arteriosus

Over time, 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. 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 usually develops before a child reaches puberty. However, it also can develop in young adulthood.

• Abnormal heart rhythm (arrhythmia)
• Bluish lips, fingers, and toes (cyanosis)
• Chest pain
• Coughing up blood
• Dizziness
• Fainting
• Feeling tired
• Heart attack
• 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:

• Enlarged fingers and toes (clubbing)
• Murmur in the chest

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 (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.

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 whether it works is unclear. 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
• 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.

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

Eisenmenger complex; Eisenmenger disease; Eisenmenger reaction; Eisenmenger physiology

Behrman RE, Kliegman RM, Jenson HB. Behrman: Nelson Textbook of Pediatrics. 17th ed. Philadelphia, Pa: Saunders; 2004.

Updated by: Mark A Fogel, MD, FACC, FAAP, Associate Professor of Pediatrics and Radiology and Director of Cardiac MR, The Children's Hospital of Philadelphia.