Eisenmenger syndrome

Eisenmenger syndrome is a complication from congenital heart disease lesion. Patients have a congenital heart defect with 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 backwards because of worsening permanent pulmonary hypertension. Because of the reversed flow, patients 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 are repaired very late in life or were never repaired. Unrepaired heart lesions associated with Eisenmenger syndrome include:

  • Atrial septal defect: a hole between the two atria
  • Ventricular septal defect: a hole between the two ventricles
  • Atrioventricular canal defect: a large hole in the center of the heart creating a communication between the atria, ventricles, or both. There is also valve abnormalities associated with this lesion.
  • Patent ductus arteriosus: a connection between the aorta and the pulmonary artery.

The specialists at Norton Children’s Heart Institute — 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, affiliated with the University of Louisville, have the skill and experience to provide a pinpoint diagnosis and develop a customized treatment plan for you and your child.

The Society of Thoracic Surgeons has ranked Norton Children’s Heart Institute’s pediatric heart care among the best in the region. 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: For this procedure, 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 three-dimensional 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

Treating 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 Institue specialists would guide you through.

Why Norton Children’s Heart Institute?

Norton Children’s Heart Institute, affiliated with the University of Louisville, is a comprehensive pediatric heart surgery, heart failure and heart transplant program serving Kentucky, Southern Indiana and beyond.

The goal of the full-service Norton Children’s Heart Institute is to provide care for the child and the whole family. Our specialists are prepared to repair even the most complex congenital and acquired heart conditions.

Our heart team includes:

  • Pediatric cardiothoracic surgeons
  • Pediatric transplant surgeons
  • Pediatric cardiologists
    • Fetal cardiologists
    • Adult congenital heart cardiologists
    • Heart failure/heart transplant cardiologists
    • Pediatric electrophysiologists
    • Pediatric cardiac catheterization cardiologists
  • Pediatric cardiovascular anesthesiologists
  • Pediatric intensive care physicians
  • Cardiac critical care nurses
  • Critical care pharmacists
  • Family support team
  • Child life specialists
  • Rehabilitation specialists
  • Social workers
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Norton Children’s Heart Institute

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