Eisenmenger Syndrome

Also known as: ES, Eisenmenger physiology, Eisenmenger reaction.

What is Eisenmenger syndrome?

Eisenmenger syndrome is a heart problem that develops over time (frequently adolescents/young adults), as a result of other congenital (before birth) defects in the heart (usually left heart-to-right heart shunts). When a child is born with a hole between two chambers of the heart, or some other types of defect which increases blood flow to the lungs, it can cause high blood pressure to develop in the lungs (pulmonary hypertension). This causes narrowing of the blood vessels that over time leads to heart damage. When the pressure in the lung blood vessels rises high enough, the blood flow reverses, causing both insufficient blood going to the lungs (to be oxygenated), mixed oxygen-poor and oxygen-rich blood flowing out to the body.
 

What causes Eisenmenger syndrome? 

Eisenmenger syndrome develops over time because of heart defects (including atrial septal defect-ASD; patent ductus arteriosis-PDA; and ventricular septal defect-VSD; atrioventricular canal defect-AV canal) that cause long standing pulmonary hypertension.
 

What are the signs/symptoms of Eisenmenger syndrome?

 Symptoms includes bluish/grayish colored skin (cyanosis ), high pressure in the blood vessels going to the lungs, erythrocytosis (higher than normal number of red blood cells), shortness of breath, fatigue, chest pain or tightness, heart palpitations, blurred vision, headaches and dizziness/fainting etc.
 

What are Eisenmenger syndrome care options? 

The goals of treatment are to reduce the pressure in the pulmonary artery, improve oxygenation and decrease the cyanosis/increased red blood cells. Medical treatment is based on symptoms and includes medications, oxygen treatment and/or phlebotomy (in which some of the blood is removed and replaced with fluid). Heart-lung transplantation may be considered when other treatments are not effective.


Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: 6/12/2018 11:49:34 AM

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Once the cardiac team met with Teegan and her family, the doctors realized that this was a case unlike any they had seen before. From before birth she had been diagnosed with hypoplastic left heart syndrome, and then they realized that she had a little less than half a heart and only one lung. Teegan's doctors and her parents knew she had a long road to recovery, but they were prepared to do whatever it takes to get her there.