What is Eisenmenger Syndrome?

Eisenmenger Syndrome is a condition associated with a long-term, uncorrected heart defect present at birth. When someone is born with a heart defect, doctors call it a “congenital heart defect”.

Over time, the unrepaired defect changes the natural flow of blood between your heart and lungs. As a result, blood vessels in your lungs harden and narrow, intensifying pressure on your lungs. Ultimately, this process causes permanent damage to your blood vessels.

Other synonyms for Eisenmenger Syndrome:

• Eisenmenger complex
• Eisenmenger disease
• Eisenmenger physiology
• Eisenmenger reaction

Signs & Symptoms

You may notice symptoms that affect your energy, vision, head, chest, hands, and feet.

Common Eisenmenger Syndrome symptoms:

• Blurry vision
• Tightness in chest
• Chest pain
• Headache
• Dizziness
• Enlarged fingernails
• Enlarged toenails
• Tingling in fingers
• Tingling in toes
• Blue skin color
• Gray skin color
• Trouble breathing
• Fatigue
• Coughing up blood
• Numbness in fingers
• Numbness in toes
• Fainting

When to See a Doctor

All Eisenmenger Syndrome symptoms can indicate a potentially serious health condition. If you or a loved one experience any of the signs and symptoms of Eisenmenger Syndrome, a cardiologist at Baptist Health may be able to help.

Causes

Eisenmenger Syndrome is caused by a hole in the heart present at birth. Doctors do not believe that the condition is inherited. The hole in a patient’s heart allows excess blood to flow between the heart and lungs.

Left untreated, this excess blood flow increases pressure to the lungs and damages the blood vessels in the lungs. This can also lead to reduced oxygen levels in your body and a bluish discoloration of your skin.

In addition to Eisenmenger Syndrome causes, there are also risk factors for the condition. The main risk factor is a family history of heart defects.

How Eisenmenger Syndrome Develops

Eisenmenger Syndrome develops from an untreated congenital heart defect. There are several types of heart defects that can lead to the condition.

These types of defects include:

• Atrioventricular canal defect (AV canal) - This defect consists of a series of structural abnormalities inside your heart.
• Atrial septal defect (ASD) - A gap between the two upper chambers of the heart.
• Patent ductus arteriosus (PDA) - A hole between the pulmonary artery and the aorta permits blood to abnormally recirculate into the lungs.
• Ventricular septal defect (VSD) - A hole between the two lower chambers of the heart.

Diagnosis

Doctors rely on several diagnostic tests to make an Eisenmenger Syndrome diagnosis. The Eisenmenger syndrome age of onset depends on the cause and how long the underlying heart defect has gone unnoticed and untreated. Since the condition is the result of a long-term uncorrected defect, the age of onset is sometimes in the second or third decade of life.

Diagnostic tests include:

• Cardiac Catheterization - Your doctor inserts a long, skinny tube into your blood vessel and then guides it through your body to your heart. The doctor then tests the blow flow between your blood vessels and your heart.
• Echocardiography - This test uses ultrasound waves to examine the properties and function of your heart.
• Chest X-ray - Your doctor may use a chest X-ray machine to capture images of your heart and lungs.
• Pulmonary Function Test - This is a noninvasive test that measures the health and proper functioning of your lungs.
• Electrocardiogram - An electrocardiogram is also known as an ECG or EKG. The test analyzes the electrical function of your heartbeat.
• Blood Test - Your doctor may perform a complete blood test to look for indicators of Eisenmenger Syndrome.
• Iron Level Test - Deficient iron in your blood is a sign of Eisenmenger Syndrome.

Treatment & Recovery

Treating Eisenmenger Syndrome involves several treatment and management methods. There is no cure for the condition. The most common treatment for Eisenmenger syndrome is medication.

Potential treatment options:

• Medication, such as supplements, to treat iron deficiency.
• Medication to treat an abnormal heartbeat.
• Medication to lower blood pressure.
• Medication, such as antibiotics, to fight infections.
• Medication to thin your blood.
• Medication to relax the walls of blood vessels.

Surgery is usually not recommended for treating Eisenmenger Syndrome.

Your doctor will likely make additional recommendations for how to manage your condition.

Management approaches include:

• Ongoing scheduled visits with your doctor.
• Physical exams to check for symptoms and overall health.
• Blood tests to monitor your condition.
• Heart health tests to manage symptoms.

If other treatment and management options do not control your symptoms, doctors may recommend a heart and/or lung transplant.

Complications

There are certain complications that can occur with Eisenmenger Syndrome. These complications usually involve medication, pregnancy, high altitudes, and shifts in blood pressure.

Your doctor may suggest steps you can take to help prevent future problems with Eisenmenger Syndrome.

Eisenmenger Syndrome prevention:

• Ask your doctor about restrictions to your physical activity.
• Limit exposure to high-altitude environments due to lower oxygen levels.
• Stay hydrated, especially in warmer rooms or environments.
• Stop using any form of tobacco products.
• To avoid sudden drops in blood pressure, take shorter baths and showers, especially when the water is hot.
• Use warm water instead of hot water when taking baths and showers.
• Limit time in hot tubs.
• Check with your doctor before taking any medications or supplements.
• Protect yourself from infections by getting vaccinated.
• Check with your doctor before traveling by plane.
• Use contraceptives as pregnancy can be life-threatening.