Eisenmenger SyndromeSymptoms, Doctors, Treatments, Advances & More
Learn About 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.
Eisenmenger complex; Eisenmenger disease; Eisenmenger reaction; Eisenmenger physiology; Congenital heart defect - Eisenmenger; Cyanotic heart disease - Eisenmenger; Birth defect heart - Eisenmenger; Eisenmenger's 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.
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)
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.
Michael Gatzoulis practices in London, United Kingdom. Mr. Gatzoulis is rated as an Elite expert by MediFind in the treatment of Eisenmenger Syndrome. His top areas of expertise are Tetralogy of Fallot, Congenital Heart Disease (CHD), Eisenmenger Syndrome, Heart Transplant, and Cardiac Ablation.
Atrium Health Levine Children's HEARTest Yard Congenital Heart Center
Jorge Alegria is a Cardiologist in Charlotte, North Carolina. Dr. Alegria is rated as an Advanced provider by MediFind in the treatment of Eisenmenger Syndrome. His top areas of expertise are Transposition of the Great Arteries, Tetralogy of Fallot, Dextrocardia with Situs Inversus, and Situs Inversus. Dr. Alegria is currently accepting new patients.
Konstantinos Dimopoulos practices in London, United Kingdom. Mr. Dimopoulos is rated as an Elite expert by MediFind in the treatment of Eisenmenger Syndrome. His top areas of expertise are Congenital Heart Disease (CHD), Eisenmenger Syndrome, Pulmonary Hypertension, Heart Transplant, and Cardiac Ablation.
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.
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
Contact your provider if you or your child develops symptoms of Eisenmenger syndrome.
Surgery as early as possible to correct the heart defect can prevent Eisenmenger syndrome.
Summary: What is this study about? This study will test whether adding sotatercept to usual medicines for pulmonary arterial hypertension (PAH) can help adults who have PAH due to unrepaired congenital heart defects (atrial or ventricular septal defect, or patent ductus arteriosus), including Eisenmenger syndrome. These conditions often cause long-standing changes in the lung blood vessels and low oxygen l...
Summary: In view of the manifold options for mono- and combination therapy that have now emerged for patients with pulmonary (arterial) hypertension (PH/PAH), controlled clinical trials can only provide part of the information needed for optimal management. In order to gather adequate data on PAH/PH treatment in routine clinical care, the ongoing COMPERA registry prospectively documents consecutive patient...
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.
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.