Eisenmenger Syndrome

What Is Eisenmenger Syndrome?

Eisenmenger syndrome is a condition that primarily affects adolescents and adults with certain congenital heart defects that are never repaired or repaired after the first year of life.

Due to these heart defects, blood is not properly distributed throughout the body and often results in pulmonary hypertension (high blood pressure in the lungs).

Symptoms of Eisenmenger syndrome

The symptoms of Eisenmenger syndrome may resemble other medical conditions or heart problems. Always consult your doctor for a thorough evaluation. These symptoms may include, but are not limited to:

• Cyanosis (pale blue or grayish skin tone)
• Shortness of breath during activity
• Shortness of breath at rest
• Chest pain or tightness
• Headache, dizziness or fainting
• Numbness in toes or fingers
• Blurred vision

Additionally, there are complications often associated with Eisenmenger syndrome. These may include:

• Blood clots
• Hemorrhage
• Stroke
• Brain abscesses
• Gout

What causes Eisenmenger syndrome?

Eisenmenger syndrome is a condition that results from high blood pressure in the lungs (pulmonary hypertension) due to congenital heart disease.

Here are some common congenital heart diseases that can lead to Eisenmenger syndrome:

• Patent ductus arteriosus (PDA)
• Atrial septal defect (ASD)
• Ventricular septal defect (VSD)
• Atrioventricular canal defect (AV canal)

Learn more about what causes Eisenmenger syndrome.

Diagnosing Eisenmenger syndrome

Patients that are children or adolescents with a congenital heart disease that has not been repaired, or was repaired after age 1 are most at risk of developing Eisenmenger syndrome.

Along with a physical examination of the heart and lungs your doctor may utilize the following tools to help diagnose Eisenmenger syndrome:

• Blood tests
• Chest X-ray
• Electrocardiogram (EKG or ECG)
• Echocardiogram
• Cardiac catheterization
• Magnetic resonance imaging (MRI)

Treatment for Eisenmenger syndrome

The goals of treatment for Eisenmenger syndrome are aimed at increasing a patient’s quality of life.  This may include, but is not limited to:

• Decreasing blood pressure in the lungs
• Improving oxygen levels in the blood
• Decreasing cyanosis (blue/gray skin)
• Decreasing erythrocytosis (elevated red blood cell count)

A doctor will determine a course of treatment for Eisenmenger syndrome based on each patient’s medical history and their individual preferences. Additional factors may include:

• Age and overall health of patient
• Extent of disease/ rate of progression
• Patient’s tolerance of medications

Here are some treatments that are effective in helping to improve the quality of life of patients suffering from Eisenmenger syndrome:

• Phlebotomy
• Medications
• Oxygen
• Other treatments