Eisenmenger syndrome

What causes Eisenmenger syndrome?

Eisenmenger syndrome (ES) is caused by a defect in the heart. The most common heart defect that can lead to ES is a ventricular septal defect (VSD), a hole between the two pumping chambers (the left and right ventricles) of the heart. Other heart defects that can lead to Eisenmenger syndrome include atrial septal defect (ASD) and patent ductus arteriosus (PDA). These heart defects allow blood that has already picked up oxygen from the lungs to flow back into the lungs, instead of going out to the rest of the body. This can eventually lead to increased pressure in the pulmonary artery (pulmonary hypertension), the main blood vessel that connects the heart and the lungs. Increased pulmonary hypertension leads to abnormal blood flow and results in less oxygen going to the body.

Over time, this abnormal blood flow can damage the small blood vessels in the lungs. This causes high blood pressure in the lungs. As a result, the blood backs up and does not go to the lungs to pick up oxygen. Instead, the blood goes from the right side to the left side of the heart (right to left shunt), and oxygen-poor blood travels to the rest of the body.

Last updated on 05-01-20

How is Eisenmenger syndrome diagnosed?

Eisenmenger syndrome (ES) is diagnosed based on the symptoms and the presence of a heart defect. Additional blood work and other medical tests may be done to determine the extent of the symptoms. These tests may include pulse oximetry (checks blood oxygen levels), chest x-ray, EKG, pulmonary function tests, complete blood count (CBC), and a test for iron levels.

Last updated on 05-01-20

How is Eisenmenger syndrome inherited?

Eisenmenger syndrome is not inherited in families. However, genetic factors may contribute to the chance to be born with a heart defect.

Last updated on 05-01-20

What is the long-term outlook for people with Eisenmenger syndrome?

The long-term outlook for someone with Eisenmenger syndrome depends on the severity of the symptoms and the presence of any other health conditions. Typically, the symptoms of ES get worse over time.

Last updated on 05-01-20

How many people have Eisenmenger syndrome?

The exact number of people with Eisenmenger syndrome (ES) is unknown. This condition occurs in about 1-6% of adults who are born with a heart defect. ES is more common in people who have a heart defect that was not detected or surgically repaired as a child.

Last updated on 05-01-20

How might Eisenmenger syndrome be treated?

A person with Eisenmenger syndrome (ES) may need to be seen by several specialists, including a heart specialist (cardiologist), a doctor who specialize in taking care of the lungs (pulmonologist) and a doctor who takes care of blood and blood diseases (hematologist), as well as other specialists. Treatment usually involves different medications to help lessen the severity of the symptoms. Medications may include diuretics to control excess fluid, medications to help regulate heart rate, and anticoagulants to help prevent blood clots. Some people are placed on antibiotics to help reduce the chance of getting an infection. Vasodilators, medications that help relax the blood vessels may also be helpful.

In addition, people with ES are advised to avoid situations that may make their symptoms worse, including pregnancy, high altitudes, and extreme physical exercise.

Oxygen therapy has been helpful for some people, but it may not prevent the symptoms from getting worse. People with severe symptoms may need a heart-lung transplant.

Last updated on 05-01-20

Name: Congenital Heart Information Network (C.H.I.N.) P.O. Box 3397
Margate City, NJ, 08402-0397, United States
Phone: (609) 823-4507 Fax : 609-822-1574 Email: mb@tchin.org Url: https://www.facebook.com/TCHIN.org?
Name: American Heart Association 7272 Greenville Avenue
Dallas, TX, 75231-4596, United States
Phone: 214-570-5978 Toll Free: 800-242-8721 Email: https://www.heart.org/en/forms/general-questions-and-latest-research-information Url: https://www.heart.org
Name: National Heart, Lung, and Blood Institute NHLBI P.O Box 30105
Bethesda, MD, 20854-0105,
Phone: 301-592-8573 Email: nhlbiinfo@nhlbi.nih.gov Url: https://www.nhlbi.nih.gov
Wallen TJ, Sergent BN. Eisenmenger Syndrome In: StatPearls [Internet]. Updated Oct 27, 2018; Reference Link Connolly HM. Management of Eisenmenger syndrome UpToDate. Apr 26, 2018; Reference Link Connolly HM. Evaluation and prognosis of Eisenmenger syndrome UpToDate. May 1, 2018; Reference Link Clave MM, Maeda NY, Castro CRP, Bydlowski SP, Lopes AA. Factors influencing outcomes in patients with Eisenmenger syndrome: a nine-year follow-up study. Pul Circ. 2017; 7(3). 635-642. Reference Link Celermajer DS. Eisenmenger syndrome: a rare malady that continues to fascinate Eur Heart Jl. 2017; 38. 2068-2069. Reference Link

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