Eisenmenger syndrome

By Medically reviewed by hellodoktor

Definition

What is Eisenmenger syndrome?

Eisenmenger syndrome is a complication of a heart defect that you’re born with (congenital).

A heart defect that causes a hole (shunt) to develop between two chambers of your heart is the most common cause of Eisenmenger syndrome. This hole causes blood to circulate abnormally in your heart and lungs. Increased blood flow returns to your lungs instead of going to the rest of your body. The blood vessels in your lung arteries become stiff and narrow, increasing the pressure in your lungs’ arteries. This permanently damages the blood vessels in your lungs.

Eisenmenger syndrome occurs when the increased pressure of the blood flow in the lung becomes so great that the direction of blood flow through the shunt reverses. Oxygen-poor (blue) blood from the right side of the heart flows into the left ventricle and is pumped to your body so you don’t receive enough oxygen to all your organs and tissues.

Eisenmenger syndrome is a life-threatening condition requiring careful medical monitoring. Medications can improve symptoms and prognosis.

How common is Eisenmenger syndrome?

Eisenmenger syndrome is rare. Eisenmenger syndrome appears to affect males and females in relatively equal numbers. Please discuss with your doctor for further information.

Symptoms

What are the symptoms of Eisenmenger syndrome?

The common symptoms of Eisenmenger syndrome are:

  • Bluish or grayish skin color (cyanosis)
  • Large, rounded fingernails or toenails (clubbing)
  • Easily tiring and shortness of breath with activity
  • Shortness of breath while at rest
  • Chest pain or tightness
  • Skipped or racing heartbeats (palpitations)
  • Fainting (syncope)
  • Coughing up blood (hemoptysis)
  • Dizziness
  • Numbness or tingling in the fingers or toes
  • Headaches
  • Abdominal swelling

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you have any signs or symptoms listed above or have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes Eisenmenger syndrome?

Eisenmenger syndrome is a kind of high blood pressure in the lungs, called pulmonary arterial hypertension. It’s caused by a heart defect that lets blood from opposite sides of your heart mix together. Usually, this happens when there’s a hole between the two lower chambers of your heart, called the ventricles, or a hole between the upper chambers, called the atria.

The right side of your heart pumps blood a short distance to your lungs, where it picks up oxygen. The left side sends blood out to the rest of your body, so it pumps much harder. When there’s an opening between the two sides, the stronger left side forces blood into the right side. That sends too much blood into your lungs.

Over time, the blood vessels of your lungs are damaged. They get stiff and thick, and the pressure inside them rises. Eventually, pressure also rises in the right side of your heart. That forces blood from the right side into the left, so blood that hasn’t made it to the lungs yet is mixed with the oxygen-rich blood being pumped out to your body. Organs and tissues don’t get enough oxygen, and that causes serious problems.

This damage happens slowly, and symptoms can take many years to show up. The process starts before a child turns 2, and by the teenage years, blood starts to flow the opposite way through the hole in the heart and oxygen levels drop. Health problems start in the teens and 20s.

Risk factors

What increases my risk for Eisenmenger syndrome?

Please discuss with your doctor for further information.

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is Eisenmenger syndrome diagnosed?

Your doctor may notice a problem when he listens to your heart. He’ll look for signs of a heart defect and low levels of oxygen in your blood. He’ll put a sensor on your finger or earlobe to measure how much oxygen is in your blood.

He may also take a sample of your blood to see if you have more red blood cells than normal. They’re the part of your blood that carries oxygen. When your body doesn’t get enough oxygen, it makes extra red blood cells.

Your doctor can confirm that you have Eisenmenger syndrome with a chest X-ray or echocardiogram, which can show if there are holes between the chambers. He might also suggest an electrocardiogram (EKG), which checks for problems with the electrical activity of your heart.

How is Eisenmenger syndrome treated?

Once blood vessels in your lungs are damaged, heart surgery alone can’t fix the problem. Some people can have a lung transplant. Others get a transplant of both heart and lungs.

But surgery is very risky, and it can be hard to find donor organs. If surgery isn’t an option, your doctor will treat your symptoms and try to prevent other health issues. Certain drugs can bring down the blood pressure in your lungs and limit or delay damage to your heart and blood vessels.

Other medications can help with specific symptoms, like iron supplements for anemia or diuretics to help with swelling. Oxygen may make you feel better.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage Eisenmenger syndrome?

The following lifestyles and home remedies might help you cope with Eisenmenger syndrome:

  • Check with your doctor about exercise restrictions. While you shouldn’t perform strenuous exercise or sports, you may be able to do less intense physical activities. Talk to your doctor about what type of physical activity is appropriate for you.
  • Avoid high altitudes. Because of the low oxygen levels at high altitudes, the American College of Cardiology and the American Heart Association recommend against living at an altitude of 5,000 feet (1,524 meters) or higher above sea level. Discuss travel by airplane or to high altitudes with your cardiologist for specific recommendations.
  • Avoid situations that can excessively lower blood pressure. These include sitting in a hot tub or sauna or taking long hot baths or showers. These activities lower your blood pressure and cause fainting or even death. You should also avoid activities that cause prolonged straining, such as lifting heavy objects or weights.
  • Be cautious with any medications and supplements. Many prescription and over-the-counter medications or supplements may increase or decrease blood pressure, increase risk of bleeding or blood clots, or affect kidney function in patients who have Eisenmenger syndrome. Talk to your doctor before taking any supplements or medications.
  • Avoid secondhand smoke and quit using tobacco products. Cigarette smoke and other tobacco products can cause further damage to your lungs’ arteries and increase your risk of developing complications.

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

Sources

Review Date: October 9, 2017 | Last Modified: September 11, 2019

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