Learn About Eisenmenger Syndrome

What is the definition of Eisenmenger Syndrome?

Eisenmenger syndrome is a condition that affects blood flow from the heart to the lungs in some people who were born with structural problems of the heart.

What are the alternative names for Eisenmenger Syndrome?

Eisenmenger complex; Eisenmenger disease; Eisenmenger reaction; Eisenmenger physiology; Congenital heart defect - Eisenmenger; Cyanotic heart disease - Eisenmenger; Birth defect heart - Eisenmenger; Eisenmenger's syndrome

What are the causes of Eisenmenger Syndrome?

Eisenmenger syndrome is a condition that results from abnormal blood circulation caused by a defect in the heart. Most often, people with this condition are born with a hole between the two larger pumping chambers -- the left and right ventricles -- of the heart (ventricular septal defect). The hole allows 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.

Other heart defects that can lead to Eisenmenger syndrome include:

  • Atrioventricular canal defect
  • Atrial septal defect
  • Cyanotic heart disease
  • Patent ductus arteriosus
  • Truncus arteriosus

Over many years, increased blood flow can damage the small blood vessels in the lungs. This causes high blood pressure in the lungs. As a result, the blood flow goes backward through the hole between the two pumping chambers. This allows oxygen-poor blood to travel to the rest of the body.

Eisenmenger syndrome may begin to develop before a child reaches puberty. However, it also can develop in young adulthood, and may progress throughout young adulthood.

What are the symptoms of Eisenmenger Syndrome?

Symptoms include:

  • Bluish lips, fingers, toes, and skin (cyanosis)
  • Rounded fingernails and toenails (clubbing)
  • Numbness and tingling of fingers and toes
  • Chest pain
  • Coughing up blood
  • Dizziness
  • Fainting
  • Feeling tired
  • Shortness of breath
  • Skipped heartbeats (palpitations)
  • Stroke
  • Swelling in the joints caused by too much uric acid (gout)
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What are the current treatments for Eisenmenger Syndrome?

Affected people may receive oxygen, although it is unclear if it helps to prevent the disease from getting worse. In addition, medicines that work to relax and open the blood vessels may be given. People with very severe symptoms may eventually need a heart-lung transplant.

Who are the top Eisenmenger Syndrome Local Doctors?

Practice Associates Medical Group

100 Madison Ave, 
Morristown, NJ 
 59.4 mi
Offers Telehealth

Claire Boccia-Liang is a Cardiologist in Morristown, New Jersey. Dr. Boccia-Liang and is rated as an Advanced provider by MediFind in the treatment of Eisenmenger Syndrome. Her top areas of expertise are Total Anomalous Pulmonary Venous Return, Arrhythmias, Atrial Fibrillation, and Sick Sinus Syndrome.

Family Medicine
Family Medicine
563 Westfield Ave, 
Westfield, NJ 
 65.7 mi

Claudia Wagner is a Family Medicine provider in Westfield, New Jersey. Dr. Wagner and is rated as an Advanced provider by MediFind in the treatment of Eisenmenger Syndrome. Her top areas of expertise are Eisenmenger Syndrome, High Cholesterol, Obesity in Children, and Partial Atrioventricular Canal.

 
 
 
 
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Family Medicine | General Practice | Internal Medicine
Family Medicine | General Practice | Internal Medicine
1163 Old Country Rd, 
Plainview, NY 
 57.2 mi
Offers Telehealth

Dorit Ben-Moha is a Family Medicine specialist and an Internal Medicine provider in Plainview, New York. Dr. Ben-Moha and is rated as an Advanced provider by MediFind in the treatment of Eisenmenger Syndrome. Her top areas of expertise are Sepsis, Pneumonia, Painful Swallowing, Gastrostomy, and Ureteroscopy.

What is the outlook (prognosis) for Eisenmenger Syndrome?

How well the affected person does depends on whether another medical condition is present, and the age at which high blood pressure develops in the lungs. People with this condition can live 20 to 50 years.

What are the possible complications of Eisenmenger Syndrome?

Complications may include:

  • Bleeding (hemorrhage) in the brain
  • Congestive heart failure
  • Gout
  • Heart attack
  • Hyperviscosity (sludging of the blood because it is too thick with blood cells)
  • Infection (abscess) in the brain
  • Kidney failure
  • Poor blood flow to the brain
  • Stroke
  • Sudden death
When should I contact a medical professional for Eisenmenger Syndrome?

Contact your provider if you or your child develops symptoms of Eisenmenger syndrome.

How do I prevent Eisenmenger Syndrome?

Surgery as early as possible to correct the heart defect can prevent Eisenmenger syndrome.

What are the latest Eisenmenger Syndrome Clinical Trials?
Pentoxifylline as an Adjunct Therapy for Patients With Eisenmenger Syndrome: a Randomized Study

Summary: The Eisenmenger syndrome corresponds to the most advanced form of pulmonary arterial hypertension associated with congenital heart disease. The syndrome causes chronic hypoxemia, with an increase in erythrocyte mass, which predisposes to thrombotic complications. Pentoxifylline is a xanthine derivative and it is considered as a hemorrheological agent with described effects of reduction in erythroc...

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Influence of Respiratory and Exercise Therapy on Oxygen Uptake, Quality of Life in Patients With Severe Associated Pulmonary Arterial Hypertension (APAH) as Part of a Congenital Heart Defect With / Without Eisenmenger's Syndrome

Summary: The purpose of this study is to investigate the influence of physical training on exercise capacity, quality of life, functional class, oxygen consumption and right ventricular function in patients with severe associated pulmonary arterial hypertension (APAH) as part of a congenital heart defect with / without Eisenmenger's Syndrome

Who are the sources who wrote this article ?

Published Date: February 27, 2024
Published By: Thomas S. Metkus, MD, Assistant Professor of Medicine and Surgery, Johns Hopkins University School of Medicine, Baltimore, MD. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

Bernstein D. General principles of treatment of congenital heart disease. In: Kliegman RM, St. Geme JW, Blum NJ, et al, eds. Nelson Textbook of Pediatrics. 22nd ed. Philadelphia, PA: Elsevier; 2025:chap 483.

Therrien J, Marelli AJ. Congenital heart disease in adults. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 55.

Valente AM, Dorfman AL, Babu-Narayan SV, Krieger EV. Congenital heart disease in the adolescent and adult. In: Libby P, Bonow RO, Mann DL, Tomaselli GF, Bhatt DL, Solomon SD, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 12th ed. Philadelphia, PA: Elsevier; 2022:chap 82.