What is the definition of 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.

What are the alternative names for Eisenmenger Syndrome?

Eisenmenger complex; Eisenmenger disease; Eisenmenger reaction; Eisenmenger physiology; Congenital heart defect - Eisenmenger; Cyanotic heart disease - Eisenmenger; Birth defect heart - Eisenmenger

What are the causes for Eisenmenger Syndrome?

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.

Other heart defects that can lead to Eisenmenger syndrome include:

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

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 flow goes backward through the hole between the two pumping chambers. This allows 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.

What are the symptoms for Eisenmenger Syndrome?

Symptoms include:

• Bluish lips, fingers, toes, and skin (cyanosis)
• Rounded fingernails and toenails (clubbing)
• Numbness and tingling of fingers and toes
• Chest pain
• Coughing up blood
• Dizziness
• Fainting
• Feeling tired
• Shortness of breath
• Skipped heartbeats (palpitations)
• Stroke
• Swelling in the joints caused by too much uric acid (gout)

What are the current treatments for Eisenmenger Syndrome?

At times, people with symptoms may have blood removed from the body (phlebotomy) to reduce the number of red blood cells. The person then receives 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. In addition, medicines that work to relax and open the blood vessels may be given. People with very severe symptoms may eventually need a heart-lung transplant.

What is the outlook (prognosis) for Eisenmenger Syndrome?

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. People with this condition can live 20 to 50 years.

What are the possible complications for Eisenmenger Syndrome?

Complications may include:

• 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 should I contact a medical professional for Eisenmenger Syndrome?

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

How do I prevent Eisenmenger Syndrome?

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

REFERENCES

Bernstein D. General principles of treatment of congenital heart disease. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 461.

Therrien J, Marelli AJ. Congenital heart disease in adults. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 61.

Webb GD, Smallhorn JF, Therrien J, Redington AN. Congenital heart disease in the adult and pediatric patient. In: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 11th ed. Philadelphia, PA: Elsevier; 2019:chap 75.