Eisenmenger Syndrome

Eisenmenger syndrome occurs as a complication of certain congenital heart defects. It is characterized by a reversal of blood flow within the heart and pulmonary arteries, leading to increased pressure in the lungs (pulmonary hypertension). 

 

Causes

Eisenmenger syndrome occurs when an unrepaired/partially repaired congenital heart defect (ie. ASD, VSD, PDA) that causes abnormal blood flow between the heart chambers leads to increased pressure in the lungs. Over time, the increased pressure damages the blood vessels in the lungs, causing them to thicken and narrow. As a result, blood flow reverses, with oxygen-poor blood mixing with oxygen-rich blood, and this leads to decreased oxygen supply to the body.

 

Symptoms

Symptoms of Eisenmenger syndrome may vary depending on the severity of the condition and can include:

  • Cyanosis (bluish discoloration of the skin, lips, and nails) due to decreased oxygen levels in the blood
  • Shortness of breath, especially during physical activity or exertion
  • Fatigue and weakness
  • Fainting or dizziness
  • Chest pain or discomfort
  • Rapid heartbeat or palpitations
  • Clubbing of fingers and toes (enlargement and rounding of the nail beds)

 

Diagnosis

To diagnose Eisenmenger syndrome, the following tests and assessments may be conducted:

  • Echocardiogram: This ultrasound test provides detailed images of the heart and can identify congenital heart defects and assess the severity of pulmonary hypertension.
  • Electrocardiogram (ECG): This test records the electrical activity of the heart and can detect any abnormal heart rhythms.
  • Cardiac catheterization: In this procedure, a thin tube is inserted into a blood vessel and guided to the heart to measure pressures in the heart chambers and blood vessels, and to assess the severity of the condition.
  • Blood tests: Blood tests may be done to evaluate oxygen levels and assess organ function.
  • Advanced cardiac imaging may be recommended.

 

Treatment

The goal of treating Eisenmenger syndrome is to manage symptoms, improve quality of life, and prevent complications. Treatment options may include:

  • Medications: Medications can help manage symptoms and reduce complications associated with Eisenmenger syndrome, such as pulmonary hypertension or heart failure.
  • Oxygen therapy: Supplemental oxygen may be prescribed to increase oxygen levels in the blood and relieve symptoms.
  • Blood thinners: In some cases, blood-thinning medications may be prescribed to reduce the risk of blood clots.
  • Heart and lung transplantation: In severe cases, heart and/or lung transplantation may be considered when other treatments are no longer effective.

 

When to consult a physician

Eisenmenger Syndrome patients should be monitored life long, by your ACHD physician. Early diagnosis and management of Eisenmenger syndrome are crucial for improving outcomes and quality of life. A UTHealth Houston ACHD physician who specializes in congenital heart conditions can provide an accurate diagnosis, recommend appropriate treatment options, and monitor your condition over time.