Eisenmenger Syndrome in Children
What is Eisenmenger syndrome in children?
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
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.
What causes Eisenmenger syndrome in a child?
Eisenmenger syndrome develops over time. It is a result of high blood pressure in the
lungs related to certain heart problems that are present at birth (congenital). Problems
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.
What are the symptoms of Eisenmenger syndrome in a child?
The following are the most common symptoms of Eisenmenger syndrome:
- Blue or grayish skin from less oxygen in the blood (cyanosis)
- Shortness of breath with activity
- Shortness of breath at rest
- Feeling tired
- Chest pain or chest tightness
- Skipping or racing heart beats
- Dizziness or fainting (syncope)
- Numbness or tingling of fingers and toes, or both
- Blurred vision
- Changes in the fingers and toes (clubbing)
- Enlarged liver
How is Eisenmenger syndrome diagnosed in a child?
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.
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:
oximetry. A small probe is used to check the amount of oxygen in the
tests. Tests may be done to check for a high or low red blood cell
- Chest X-ray.
A chest X-ray shows the heart and lungs. There may be changes in the lungs
because of extra blood flow.
- Electrocardiogram (ECG). An ECG records the electrical activity of the
heart. It also shows abnormal rhythms and detects heart muscle stress.
- Echocardiogram (echo). An echo uses sound waves to make a moving picture of
the heart and heart valves. An echo can show the direction of blood flow. It can find
out the size of a defect. Different types of echo tests may be done.
(lung) function testing. Lung function testing checks how well your child's
- Cardiac cath
(catheterization). A cardiac cath gives
very detailed information about the structures inside the heart. The doctor puts a
small flexible tube (catheter) into a blood vessel in the groin. The doctor guides
the catheter to the heart. He or she measures blood pressure and oxygen. In some
cases, the doctor may inject contrast dye to more clearly see the blood flow and
structures inside the heart.
- Cardiovascular MRI (CMRI). This test makes detailed images of the heart and
blood vessels. MRI may be used to check the amount and direction of blood
- CT scan and
CT angiography. These tests may be used to check for blood clots in the lung
arteries and lung.
test. This is an ECG done while the child is exercising.
How is Eisenmenger syndrome treated in a child?
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:
Your child will be given medicines that dilate blood vessels and lower blood
pressure in the lungs.
Your child will get oxygen while sleeping or resting, or continuously.
- Phlebotomy. The doctor may remove some blood if your child has a high red
blood cell count and thickened blood.
therapies. Other medicines are available that dilate or relax the arteries
in the lungs.
transplant or heart-lung transplant. These may be
considered in some cases.
What are possible complications of
Eisenmenger syndrome in a child?
Complications of this condition may include:
clots that may be in the deep veins in the legs (deep vein thrombosis) or lungs
- Coughing up blood (hemoptyis)
material in the brain (abscess)
- Irregular heart rhythms (arrhythmias)
- Kidney dysfunction
- Infections of heart valves (endocarditis)
How can I help my child live with Eisenmenger syndrome?
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:
- Anesthesia and surgery are considered high risk and should be carefully planned. If your child needs surgery, the surgeon and other healthcare providers should work with your child's pediatric cardiologist.
child should not go to high altitudes. Your child may be able to fly as long as the
plane is pressurized. Using oxygen during flights may also decrease the chance of
- Your child should not smoke or drink.
your child from things that put your child at higher risk. These include dehydration,
exercise, excessive heat, and iron deficiency.
or prevent coughing with cough-suppressing medicines. This helps lower the risk of
bleeding from the lungs.
your child get a flu shot every year. Talk with your child's healthcare provider
about the pneumococcal vaccine and other important vaccines.
for counseling your older teen or young adult if they are considering pregnancy.
The syndrome puts both mother and child at great risk. To prevent pregnancy, you
teen can use progestin-only birth control. This doesn't raise the risk for blood
Talk with your child's healthcare provider about the specific long-term outlook for your child, teen, or young adult with Eisenmenger syndrome.
When should I call my child's healthcare provider?
Symptoms that get worse or new symptoms can be early warning signs. Call your child's
healthcare provider as soon as possible.
or your local emergency number if your child
has any severe symptoms. These include:
- Serious bleeding
- Severe headache
- Loss of consciousness
Key points about Eisenmenger syndrome in children
- Eisenmenger syndrome means that the arteries that carry blood from the heart to the lungs are narrowed. This puts too much pressure on the walls of the arteries and damages the lungs.
- The condition mostly affects teens and young adults with certain congenital heart defects that are not repaired or repaired late.
- Treatment for Eisenmenger syndrome aims to lower the pressure in the pulmonary
artery, bring more oxygen to lung tissues, and decrease bluish skin and nails
- It is
important to work with your child's healthcare team to reduce the risk for
Tips to help you get the most from a visit to your child’s healthcare provider:
- Know the reason for the visit and what you want to happen.
- Before your visit, write down questions you want answered.
- At the
visit, write down the name of a new diagnosis, and any new medicines, treatments, or
tests. Also write down any new instructions your provider gives you for your
- Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.
- Ask if your child’s condition can be treated in other ways.
- Know why a test or procedure is recommended and what the results could mean.
- Know what to expect if your child does not take the medicine or have the test or procedure.
- If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
how you can contact your child’s provider after office hours. This is important if
your child becomes ill and you have questions or need advice.
Online Medical Reviewer:
Online Medical Reviewer:
Pat F Bass MD MPH
Online Medical Reviewer:
Scott Aydin MD
Date Last Reviewed:
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