Eisenmenger syndrome is an advanced form of pulmonary artery hypertension. In this condition, the arteries that carry blood from the heart to the lungs narrow. This makes the pressure of blood flow against the walls of the arteries (blood pressure) too high. The heart must work harder to pump blood into the lungs. This causes lung damage.
Eisenmenger syndrome mostly affects teens and adults with certain heart defects. It can also affect children, although it is not as common. When the heart defects are not repaired or repaired later in life, it increases the risk for this condition. In some people, it may also occur even when the defect is repaired.
Eisenmenger syndrome develops over time. It is a result of high blood pressure in the lungs related to certain heart defects present at birth (congenital). Defects more likely to cause this are those where the blood flows from the left side of the heart to the right side of the heart (left-to-right shunt).
Eisenmenger syndrome is more likely in larger heart defects.
The following are the most common symptoms of Eisenmenger syndrome:
Your child's healthcare provider will ask about your child’s symptoms. The provider may see signs when he or she examines your child. Your provider may hear an abnormal heart sound when listening to your child's heart with a stethoscope.
Your child may be referred to a pediatric cardiologist. This is a doctor with special training to treat heart problems in babies and children.
Your child may need tests. These may include:
Treatment will depend on your child’s symptoms, age, and general health. It will also depend on how severe the condition is.
Treatment focuses on lowering the pressure in the pulmonary artery. It also aims to bring more oxygen to the lung tissues and ease the cyanosis.
Medical treatment helps manage symptoms. Treatments include:
Complications of this condition may include:
Some children with Eisenmenger syndrome may live into middle adulthood. A few may live into their 50s or 60s.
Recommendations for children with Eisenmenger syndrome include:
Talk with your child's healthcare provider about the specific long-term outlook for your child, teen, or young adult with Eisenmenger syndrome.
Worsening of symptoms or new symptoms can be early warning signs. Call your child's healthcare provider as soon as possible.
Call 911 or your local emergency service if your child has any severe symptoms. These include:
Tips to help you get the most from a visit to your child’s healthcare provider:
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