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Alternative Names Return to top
Eisenmenger complex; Eisenmenger disease; Eisenmenger reaction; Eisenmenger physiologyDefinition Return to top
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.
Causes Return to top
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:
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.
Symptoms Return to top
Exams and Tests Return to top
The doctor will examine the child. During the exam, the doctor may find:
The doctor will diagnose Eisenmenger syndrome by looking at the patient’s history of heart problems. Tests may include:
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.
Treatment Return to top
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.
Outlook (Prognosis) Return to top
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.
Possible Complications Return to top
When to Contact a Medical Professional Return to top
Call your health care provider if your infant develops symptoms of Eisenmenger syndrome.
Prevention Return to top
Surgery as early as possible to correct the heart defect can prevent Eisenmenger syndrome.References Return to top
Behrman RE, Kliegman RM, Jenson HB. Behrman: Nelson Textbook of Pediatrics. 17th ed. Philadelphia, Pa: Saunders; 2004.
Update Date: 2/29/2008 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.
