Eisenmenger Syndrome

What is Eisenmenger Syndrome?

Eisenmenger syndrome is a severe complication of a congenital heart defect. It most often strikes teens and adults, but it can also afflict children. It is usually caused by a gradually developing hole in the heart that causes abnormal blood flow in the heart and lungs. As a result, the blood vessels in the lungs become narrow, stiff and irreparably damaged.

The condition puts a tremendous amount of pressure on the arteries in the lungs. When the blood pressure in the lungs becomes too great, blood flow reverses and pumps oxygen-poor blood from the right heart ventricle to the left heart ventricle before entering the body. When the blood lacks sufficient oxygen, tissues and major organs can become seriously damaged.

What are the Symptoms of Eisenmenger Syndrome?

The symptoms of Eisenmenger syndrome may include:

  • Ashen or blue complexion
  • Nail clubbing (large rounded nails that extend over the ends of fingers or toes)
  • Weakness and/or shortness of breath with physical exertion
  • Breathing problems while inactive
  • Tightness or pain in chest
  • Heart palpitations
  • Fainting
  • Blood comes up when coughing
  • Dizziness
  • Tingling or numbness in toes and fingers
  • Head pain
  • Swollen abdomen

Eisenmenger Syndrome Causes

Eisenmenger syndrome is caused by a heart defect that causes abnormal blood circulation. The defect that causes this varies, but it is most often a hole that exists between the heart’s two pumping chambers. When a hole is present between the right and left ventricles, it lets originated blood back into the lungs. A normal heart would let that blood go to the rest of the body instead.

This condition can also be caused by a number of other heart defects and diseases. Among these are an atrial septal defect, an atrioventricular canal defect, patent ductus arteriosus, cyanotic heart disease and truncus arteriosus. When this defect or disease has been present for many years, the increased flow of blood can cause damage to the lungs’ blood vessels. When that damage occurs, the blood gets backed up and no longer travels to the lungs to become oxygenated. The blood instead flows from the right side of the heart to the left and lets blood that is poor in oxygen go out to the body.

Generally, the heart problem that causes Eisenmenger syndrome is one that a baby is born with. Though it may be diagnosed later in childhood, the original heart defect was there at birth.

How is Eisenmenger Syndrome Treated?

When heart defects are not discovered and/or go unrepaired at birth, the risk of Eisenmenger syndrome greatly increases. Even early repair does not always prevent Eisenmenger syndrome. Treatment depends on age, overall health and the severity of complications, but the goals are to reduce pulmonary artery pressure and sending more oxygen to lung tissues.

Treatment includes

  • Medication to decrease blood pressure in pulmonary arteries
  • Blood thinner
  • Medication to dilate narrow blood vessels in the lungs
  • Antibiotics prior to surgical dental and medical procedures
  • Supplemental iron
  • Oxygen therapy
  • Blood draw (phlebotomy) to lower red blood cell count
  • Irreversible birth control methods to prevent life-threatening pregnancies
  • Heart-lung transplant or heart repair when treatments fail

Surgeons do not recommend heart repair as a solution for those with Eisenmenger syndrome because of greatly heightened risks. Those with Eisenmenger syndrome typically live into their 50’s or 60’s, but some survive longer with regular monitoring and proper care.

Eisenmenger Syndrome Prevention

Becuase it is a congenital defect that causes this syndrome, there is no way to prevent that defect from occurring. However, the syndrome that results from the defect can be prevented by heart surgery to fix the problem that the child was born with. Getting the surgery as early as possible can prevent much of the damage that can occur from the defect. If there is any heart irregularity in a child, or they have chest pains, dizziness or fainting, they can be tested for heart defects and can get the needed surgery before the problem progresses.

Last Reviewed:
September 20, 2016
Last Updated:
December 19, 2017