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Eisenmenger's Syndrome

What is Eisenmenger’s syndrome?

Eisenmenger’s syndrome is a condition that includes a collection of symptoms: cyanosis (pale blue or grayish skin due to decreased oxygen in the blood), pulmonary hypertension (high blood pressure in the blood vessels of the lungs), and erythrocytosis (increased number of red blood cells in the blood due to decreased oxygen in the blood).

Eisenmenger’s syndrome primarily affects adolescents and adults with certain congenital heart defects that are repaired late (after the first year of life) or that are never repaired. Eisenmenger’s syndrome, however, can occur in infants or children born with pulmonary hypertension; therefore, symptoms related to cyanosis may occur much earlier in life.

What causes Eisenmenger’s syndrome?

Eisenmenger’s syndrome progresses over time as a result of the effects of high blood pressure in the lungs. This high blood pressure, or pulmonary hypertension, occurs because of congenital heart defects that cause blood flow from the left side of the heart to the right side of the heart (left-to-right shunt). Congenital heart defects of this type include:

Because the pressures within the left side of the heart are normally greater than those within the right side of the heart, an opening between the left and right side of the heart will cause blood to flow from the left side of the heart into the right side. This left-to-right shunting of blood within the heart causes increased blood flow in the blood vessels of the lungs. The increased blood flow in the lungs’ blood vessels causes increased pressure in these vessels (pulmonary hypertension).

If the pulmonary hypertension continues without treatment, the pressure in the right side of the heart may increase to the point that the right side pressure is greater than the left. When this occurs, blood will flow from the right side of the heart to the left (right-to-left shunt), which means that oxygen-poor blood is mixed with the oxygen-rich blood pumped out to the body from the left ventricle.

A reversal of the shunt resulting in a right-to-left shunt causes insufficient oxygen in the blood. In addition, high pressure in the lungs causes progressive changes in the pulmonary blood vessels which result in irreversible damage to the lining of these blood vessels. Fibrosis (the growth of scar tissue due to infection, inflammation, injury, or even healing), and thrombus (clotting) may also occur. The changes inside the lung blood vessels may be referred to as pulmonary vascular obstructive disease or secondary pulmonary arterial hypertension (PAH). 

What are the symptoms of Eisenmenger’s syndrome?

The following are the most common symptoms of Eisenmenger’s syndrome. However, each child may experience symptoms differently, and each individual may have wide variation of symptoms from mild to severe. Symptoms may include:

Many of these symptoms can be early warning signs of changes in condition and should be regarded seriously. Symptoms should be evaluated by your child’s physician as soon as possible.

The symptoms of Eisenmenger’s syndrome may resemble other medical conditions or heart problems. Always consult your child's physician for a diagnosis.

How is Eisenmenger’s syndrome diagnosed?

A child or adolescent with a history of an atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA), or atrioventricular canal defect (AV canal) that has been unrepaired or repaired after one year of age is at risk for developing Eisenmenger’s syndrome.

The physician may note a change or increase in intensity of a cardiac murmur (a blowing or rasping sound heard while listening to the heart that may or may not indicate problems within the heart or circulatory system) during a routine physical examination. Complaints of change in exercise tolerance or any associated shortness of breath, chest pain, or palpitations, especially with activity, should be reported to the physician for further investigation.

The physician will perform a physical examination, listening to the heart and lungs, and make other observations that help in the diagnosis. Other tests that may be performed to help with the diagnosis include the following:

Treatment for Eisenmenger’s syndrome:

Specific treatment for Eisenmenger’s syndrome will be determined by your child's physician based on:

The goals of treatment for Eisenmenger’s syndrome are aimed at decreasing the pulmonary artery pressure, improving oxygenation, and decreasing degree of cyanosis and erythrocytosis. Treatment methods may include, but are not limited to, the following:

Long-term outlook with Eisenmenger’s syndrome:

Some individuals with Eisenmenger’s syndrome who have no other health complications may live into middle adulthood, and a few may survive into the fifth or sixth decade of life.

Considerations related to Eisenmenger’s syndrome include, but are not limited to, the following:

Consult your child's physician regarding the specific outlook for your child.

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