Symptoms and causes

Eisenmenger syndrome signs and symptoms include:

  • 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

Eisenmenger syndrome develops most often due to a hole between the chambers of your heart. To understand how Eisenmenger syndrome affects your heart and lungs, it's helpful to know how your heart works.

Without proper treatment and monitoring, you can develop complications of Eisenmenger syndrome, including:

  • Low oxygen levels in your blood (cyanosis). The reversed blood flow through your heart lowers the amount of oxygen your body's tissues and organs receive. This causes you to have a lower tolerance for physical activity and your skin to have a bluish or a grayish color. Cyanosis will worsen over time.
  • High red blood cell count (erythrocytosis). Because you aren't getting enough oxygen-rich blood circulating throughout your body, your kidneys release a hormone that increases your number of red blood cells, the cells that carry oxygen throughout your body.

    Having too many red blood cells can reduce the blood flow to other organs and increase your risk of developing blood clots.

  • Arrhythmias. Enlargement and thickening of the walls in the heart, along with low oxygen levels, may cause an irregular heart rhythm (arrhythmia).

    Some types of arrhythmias can cause blood to pool in your heart's chambers, where it can clot. If the clot travels out of your heart and blocks an artery, you can have a heart attack or stroke.

  • Sudden cardiac arrest. If you develop an arrhythmia as a complication of Eisenmenger syndrome, it's possible the arrhythmia could suddenly stop your heartbeat.

    Sudden cardiac arrest is the sudden, unexpected loss of heart function, breathing and consciousness. Without immediate medical attention, you can die of sudden cardiac arrest in minutes.

    You can also go into cardiac arrest during surgical procedures, usually related to changes in blood pressure caused by anesthesia.

  • Heart failure. The increased pressure in your heart can cause your heart muscles to weaken, decreasing its pumping effectiveness. Eventually, this can lead to heart failure.
  • Coughing up blood (hemoptysis). Increased pressure in the lungs and problems with your blood caused by Eisenmenger syndrome can cause life-threatening bleeding into your lungs and airways, causing you to cough up blood and further lowering your blood oxygen level. Bleeding can also occur in other parts of the body.
  • Stroke. Stoke can occur when a blood clot travels from the right to left side of the heart without being filtered out by your lungs. This blood clot may then block a blood vessel in the brain, leading to a stroke.

    The high levels of red blood cells in Eisenmenger syndrome also increase your risk of blood clots and stroke.
  • Kidney problems. Low oxygen levels in your blood may lead to problems with your kidneys. This can also increase your risk of developing gout.
  • Pregnancy risks. Due to the demands pregnancy puts on a mother's heart and lungs, women who have Eisenmenger syndrome shouldn't become pregnant. Pregnancy for a woman who has Eisenmenger syndrome poses a high risk of death for both the mother and baby.

Eisenmenger syndrome is a life-threatening condition. The prognosis for people diagnosed with Eisenmenger syndrome depends on the type of congenital heart defect and other medical conditions. People who are diagnosed with Eisenmenger syndrome can survive as long as age 50 to 60 and sometimes longer.

Aug. 04, 2017
References
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