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:

• 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; valve abnormalities also are associated with this lesion
• Patent ductus arteriosus: A connection between the aorta and the pulmonary artery

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

• 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.
• The American Board of Thoracic Surgery has certified our cardiothoracic surgeons in congenital heart surgery.
• The Adult Congenital Heart Association has accredited Norton Children’s Heart Adult Congenital Heart Disease Program as the only comprehensive care center in Kentucky and Indiana treating adults born with a heart defect.
• More than 17,000 children a year visit Norton Children’s Heart Institute for advanced heart care.
• Norton Children’s Heart Institute has offices across Kentucky and Southern Indiana to bring quality pediatric heart care closer to home.
• The Jennifer Lawrence Cardiac Intensive Care Unit (CICU) at Norton Children’s Hospital is the largest dedicated CICU in Kentucky, equipped with 17 private rooms and the newest technology available for heart care.