Eisenmenger Syndrome

Eisenmenger syndrome is a complication from a congenital heart disease lesion. It happens with a congenital heart defect that includes a hole between two chambers, causing increased blood flow to the lungs (left to right shunt). Eisenmenger syndrome occurs when the increased flow to the lungs causes pulmonary hypertension (high pressure in the blood vessels of the lungs), and flow between the two chambers eventually reverses and goes backward because of worsening permanent pulmonary hypertension.

Because of the reversed flow, children with Eisenmenger syndrome have:

  • Cyanosis: Pale blue or gray-looking skin due to low oxygen levels in the blood
  • Erythrocytosis: An increase in red blood cells due to low oxygen levels in the blood
  • Pulmonary hypertension: High pressure in the blood vessels of the lungs

Teens and adults can have Eisenmenger syndrome caused by certain congenital heart defects that were repaired later in life or were never repaired. Unrepaired heart lesions associated with Eisenmenger syndrome include:

The specialists at Norton Children’s Heart Institute, affiliated with the UofL School of Medicine — the leading providers of pediatric heart care in Louisville and Southern Indiana — can help your child with Eisenmenger syndrome.

The board-certified and fellowship-trained specialists at Norton Children’s Heart Institute have the skills and experience to provide a pinpoint diagnosis and develop a customized treatment plan for you and your child.

Norton Children’s has a network of outreach diagnostic and treatment services conveniently located throughout Kentucky and Southern Indiana.

Eisenmenger Syndrome Symptoms

  • Blurred vision
  • Chest pain or chest tightness
  • Cyanosis (pale blue or grayish skin due to decreased oxygen in the blood)
  • Dizziness or fainting
  • Fatigue
  • Heart palpitations or irregular heartbeat
  • Headache
  • Numbness and/or tingling in fingers and toes
  • Shortness of breath while at rest
  • Shortness of breath with activity or exercise

Diagnosing Eisenmenger Syndrome

A cardiologist will perform a physical exam, listen to the heart and lungs, and perform tests, including:

  • Blood tests: These will be used to look for a high red blood cell count (erythrocytosis).
  • Heart catheterization: A long, thin tube is inserted into a blood vessel at the neck or groin and guided into the heart. The doctor can get more details about the heart, including pressures and oxygen levels in the heart’s four chambers and blood vessels surrounding the heart.
  • Cardiac MRI (magnetic resonance imaging): This test uses radio waves, magnets and a computer to form 3D pictures of the heart. These pictures can show structural issues, such as an enlarged ventricle).
  • Chest X-ray: This shows pictures of the heart and lungs, which can show changes in the lungs caused by extra blood flow.
  • Electrocardiogram (ECG or EKG): This test records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias) and can show heart stress.
  • Echocardiogram (echo): This test uses sound waves (ultrasound) to produce images of the heart and blood vessels’ structures on a screen. It can show heart structure and function. Norton Children’s Heart Institute has 28 tele-echo locations throughout Kentucky and Southern Indiana.

Eisenmenger Syndrome Treatment

Eisenmenger syndrome treatment focuses on:

  • Decreasing pressure in the pulmonary artery
  • Improving oxygen levels in the blood
  • Lowering high levels of red blood cells

Treatments may include:

  • Blood removal (phlebotomy): This may be done when high red blood cell counts cause the blood to be too thick. Saline solution is added to thin the blood. This is done when symptoms are severe and/or the red blood cell levels are too high.
  • Medications: These can be given to lower high pressure in the lungs.
  • Oxygen: It can help with breathing during sleep and rest.
  • Heart or lung transplant: When other treatments are no longer effective, a transplant may be considered and would depend on a large number of factors that the Norton Children’s Heart Institute specialists would guide you through.

Why Choose Norton Children’s Heart Institute

No other congenital heart surgery program in Kentucky, Ohio or Southern Indiana is rated higher by the Society of Thoracic Surgeons than the Norton Children’s Heart Institute Pediatric Cardiothoracic Surgery Program.

  • Norton Children’s Hospital has been a pioneer in pediatric cardiothoracic surgery, performing Kentucky’s first pediatric heart transplant in 1986 and becoming the second site in the United States to perform an infant heart transplant.
  • Our board-certified and fellowship-trained pediatric cardiovascular surgeons are leaders in the field as clinicians and researchers.
  • More than 5,000 children a year visit Norton Children’s Heart Institute, affiliated with the UofL School of Medicine, for advanced heart care.
  • Norton Children’s Heart Institute successfully performs more than 17,500 procedures a year.
  • The Society of Thoracic Surgeons rated Norton Children’s Heart Institute among the best in the region after studying years of our patients’ outcomes and our ability to treat a range of pediatric heart conditions, including the most severe.
  • Norton Children’s Heart Institute has satellite outpatient offices in Ashland, Bowling Green, Campbellsville, Elizabethtown, Frankfort, London, Madisonville, Murray, Owensboro, Paducah and Shepherdsville in Kentucky; as well as Corydon, Jasper, Madison and Scottsburg in Indiana; 28 tele-echocardiography locations in Kentucky and Southern Indiana; and six fetal echocardiography locations across Kentucky.
  • The American Board of Thoracic Surgery has awarded the cardiothoracic surgeons at Norton Children’s Hospital with subspecialty certification in congenital heart surgery.
  • The Jennifer Lawrence Cardiac Intensive Care Unit (CICU) is the largest dedicated CICU in Kentucky, equipped with 17 private rooms and the newest technology available for heart care.
  • Our multidisciplinary approach to pediatric heart surgery brings together our specialists in cardiothoracic surgery, cardiology, anesthesiology, cardiac critical care and other areas to create a complete care plan tailored for your child.
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