Eisenmenger's syndrome (patient information)
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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.
What are the symptoms of Eisenmenger's syndrome?
- Abnormal heart rhythm (arrhythmia)
- Bluish lips, fingers, toes, and skin (cyanosis)
- Chest pain
- Coughing up blood
- Feeling tired
- Shortness of breath
- Swelling in the joints caused by too much uric acid (gout)
What causes Eisenmenger's syndrome?
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:
- Atrioventricular canal defect
- Atrial septal defect
- Cyanotic heart disease
- Patent ductus arteriosus
- Truncus arteriosus
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. Instead, 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.
Who is at highest risk?
Like most congenital heart defects, it is unclear exactly why certain babies are born with Eisenmenger's syndrome. There is evidence to suggest that families with a history of genetic problems and other congenital heart disease may be at an increased risk for carrying and expressing the trait. Genetic testing may be performed to assist you in estimating the likelihood that any future children may be born with Eisenmenger's syndrome.
During pregnancy, expectant mothers exposed to rubella may have an increased risk in having a baby with a heart defect. Drug and alcohol exposure during pregnancy can also harm the fetus during development and result in potential birth defects.
When to seek medical care for Eisenmenger's syndrome
Call your health care provider if your infant develops symptoms of Eisenmenger syndrome.
The doctor will examine the child. During the exam, the doctor may find:
- Enlarged fingers and toes (clubbing)
- Heart murmur (an extra sound when listening to the heart)
The doctor will diagnose Eisenmenger syndrome by looking at the patient’s history of heart problems. Tests may include:
- Complete blood count (CBC)
- Chest x-ray
- MRI scan of the heart
- Putting a thin tube in an artery to view the heart and blood vessels and measure pressures (cardiac catheterization)
- Test of the electrical activity in the heart (electrocardiogram)
- Ultrasound of the heart (echocardiogram)
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, before the irreversible damage to the small lung arteries occurs.
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 it is unclear whether it helps to prevent the disease from getting worse. Children with very severe symptoms may need a heart-lung transplant.
Where to find medical care for Eisenmenger's syndrome?
What to expect (Outlook/Prognosis)?
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.
- Bleeding (hemorrhage) in the brain
- Congestive heart failure
- 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
- Sudden death
Surgery as early as possible to correct the heart defect can prevent Eisenmenger syndrome.