Eisenmenger syndrome is the development of pulmonary hypertension (high blood pressure in the lungs) due to an untreated congenital heart defect. The disease can cause complications including permanent heart and lung damage. Nonsurgical treatments focus on easing symptoms, but there is no cure.
Eisenmenger syndrome develops when too much blood flows through the arteries that carry blood to your lungs from your heart. The result is pulmonary hypertension — high blood pressure in the blood vessels of your lungs and the right side of your heart. It also leads to blood shunting between the left and right sides of your heart. This means there’s an abnormal connection that causes blood flow from the left side of your heart to the right side of your heart.
Eisenmenger syndrome is usually the result of one or more untreated congenital (present at birth) heart defects. Over time, it can lead to permanent lung damage and other complications.
Eisenmenger syndrome typically develops in children and young adults who have an uncorrected ventricular septal defect (VSD). VSD is a congenital heart defect where there’s a hole in the wall (septum) between the heart’s two bottom pumping chambers (left and right ventricles).
Eisenmenger syndrome also can result from other unrepaired congenital heart defects such as:
Children with Down syndrome may also be at a higher risk for congenital heart defects and Eisenmenger syndrome. Between 25% and 50% of adult people with Eisenmenger syndrome have Down syndrome.
Eisenmenger syndrome is rare. It occurs in 1% to 6% of adults born with a heart defect.
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Knowing how blood flows through the heart is important to understanding Eisenmenger syndrome. In a healthy heart:
But when there’s a hole in the wall between the right and left ventricles or any other structural defects in the heart, oxygen-rich blood and oxygen-poor blood can mix. As a result, too much blood flows to the lungs, resulting in pulmonary hypertension. In addition, too little oxygen-rich blood flows to the body.
Symptoms of Eisenmenger syndrome can differ depending on the heart defect and affected organs. They don’t generally occur until puberty or early adulthood. Then they slowly worsen.
The most common signs and symptoms of the condition include:
Eisenmenger syndrome can cause a range of health complications, including:
A healthcare provider typically notices signs of Eisenmenger syndrome during a physical examination. They may order the following tests to confirm the diagnosis:
Treatment for Eisenmenger syndrome is aimed at easing symptoms and preventing severe damage to the heart or lungs. Therapies may include:
By the time Eisenmenger syndrome develops, it’s usually too late to perform a surgical repair of the underlying heart defect. However, people with severe symptoms and organ damage could need a heart or lung transplant.
The only way to prevent Eisenmenger syndrome is to have congenital heart defects repaired as early in life as possible.
People with Eisenmenger syndrome have shorter life expectancies due to the wide range of complications that can accompany the disease. However, some people survive until middle age. Survival rates vary based on the severity of the symptoms and the underlying congenital heart defect.
Pregnancy can be very dangerous for people with Eisenmenger syndrome. Risks include heart failure, blood clots and sudden death. Talk to a specialist in adult congenital heart conditions about these risks if you have the disease and are considering pregnancy.
You can reduce the risk of complications from Eisenmenger syndrome by:
You also should avoid:
A note from Cleveland Clinic
Eisenmenger syndrome is high blood pressure in the blood vessels of the lungs and the right side of the heart. An untreated congenital heart defect causes high blood pressure. Symptoms tend to develop during puberty or early adulthood and may include cyanosis, exercise intolerance and heart rhythm abnormalities. Treatment is nonsurgical and focuses on easing symptoms and preventing severe heart and lung damage.
Last reviewed by a Cleveland Clinic medical professional on 07/07/2022.
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