Eisenmenger Syndrome

What is Eisenmenger syndrome?

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.

Who gets Eisenmenger syndrome?

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:

• Atrioventricular canal defect.
• Atrial septal defect.
• Cyanotic heart disease.
• Patent ductus arteriosus.
• Tetralogy of Fallot.
• Truncus arteriosus.

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.

How common is Eisenmenger syndrome?

Eisenmenger syndrome is rare. It occurs in 1% to 6% of adults born with a heart defect.

What causes Eisenmenger syndrome?

Knowing how blood flows through the heart is important to understanding Eisenmenger syndrome. In a healthy heart:

1. Right atrium: Receives deoxygenated blood from the body before pumping it to the right ventricle.
2. Right ventricle: Pumps deoxygenated blood to the lungs where it can pick up more oxygen.
3. Left atrium: Receives oxygenated blood from the lungs before pumping it to the left ventricle.
4. Left ventricle: Pumps the oxygenated blood out to the body.

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.

What are the symptoms of Eisenmenger syndrome?

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:

• Angina (chest pain).
• Arrhythmias (abnormal heartbeat).
• Clubbing (abnormally large and curved fingernails and toenails).
• Coughing up blood.
• Cyanosis (skin with a bluish or grayish color).
• Dizziness or fainting.
• Edema (swelling in the legs, ankles, feet or abdomen.
• Heart palpitations (fast, pounding or skipped heartbeats).
• Lethargy (fatigue and lack of energy).
• Shortness of breath (dyspnea).

What are the complications of Eisenmenger syndrome?

Eisenmenger syndrome can cause a range of health complications, including:

• Bleeding in the brain.
• Brain abscess due to infection.
• Cerebral vascular insufficiency (not enough blood flow to the brain).
• Gout.
• Heart attack.
• Heart failure.
• Heart infections, such as endocarditis.
• Hyperviscosity syndrome (thick blood).
• Kidney failure.
• Pregnancy complications.
• Stroke.
• Sudden death.

How is Eisenmenger syndrome diagnosed?

A healthcare provider typically notices signs of Eisenmenger syndrome during a physical examination. They may order the following tests to confirm the diagnosis:

• Blood oxygen level
• Cardiac catheterization.
• Chest X-ray.
• Complete blood count (CBC).
• Echocardiogram (echo).
• Electrocardiogram (EKG).
• Heart MRI.
• Pulmonary function testing.

How is Eisenmenger syndrome treated?

Treatment for Eisenmenger syndrome is aimed at easing symptoms and preventing severe damage to the heart or lungs. Therapies may include:

• Antibiotics: Medication to prevent bacterial infections in the heart and lungs.
• Anticoagulants: Medication to prevent blood clots.
• Diuretics: Medication to reduce fluid buildup in the body.
• Dual endothelin receptor antagonists: Medication to lower high blood pressure in the lungs.
• Iron supplements: Additional iron to treat iron-deficiency anemia.
• Supplemental oxygen: Oxygen therapy to help you breathe better.

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.

Can Eisenmenger syndrome be prevented?

The only way to prevent Eisenmenger syndrome is to have congenital heart defects repaired as early in life as possible.

What’s the outlook for people with Eisenmenger syndrome?

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.

Can I get pregnant if I have Eisenmenger syndrome?

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.

How do I take care of myself if I have Eisenmenger syndrome?

You can reduce the risk of complications from Eisenmenger syndrome by:

• Eating a sodium-controlled diet.
• Getting vaccines to avoid infections.
• Staying hydrated.
• Taking all medications and other therapies prescribed by your cardiologist.
• Taking antibiotics before having dental work or surgery.

You also should avoid:

• High altitudes.
• Saunas, steam rooms or hot tubs, which can cause sudden drops in blood pressure.
• Smoking or using tobacco products.
• Strenuous physical exercise.

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.