Eisenmenger Syndrome: Symptoms, Causes, and Treatment
Updated: 2 days ago
Medically reviewed by Dr. Baraa Alnahhal, MD · Last reviewed: October 2026
Quick answer
Eisenmenger syndrome is a long-term complication of an unrepaired heart defect present at birth, usually a hole between the lower heart chambers, that causes high blood pressure in the lung arteries and low blood oxygen. Symptoms include blue or gray skin, chest pain and shortness of breath. Medicines improve symptoms but cannot cure it, and regular specialist care is needed. [1] [2]
What the research says
According to PubMed:
It is the most severe form of pulmonary hypertension linked to heart defects. A review in Heart describes Eisenmenger syndrome as occurring in patients with large unrepaired shunts, with central cyanosis as the main clinical sign. Progressive heart failure, infections, and sudden cardiac death are the main causes of death. (Arvanitaki et al., DOI).
Specialized lung-pressure medicines help. A Canadian Journal of Cardiology review reports that advanced pulmonary arterial hypertension therapies are indicated for patients with symptoms of WHO functional class II or higher to improve functional capacity and quality of life, and that Eisenmenger syndrome is a preventable condition through early detection of shunts. (Chaix et al., DOI).
Closing the hole is avoided once the syndrome develops. An Expert Review of Cardiovascular Therapy paper states that once Eisenmenger syndrome develops, shunt closure should be avoided, and that expert care is essential to prevent disease progression. (Barradas-Pires et al., DOI).
Study details are from PubMed abstracts.
Key facts at a glance
Fact | Detail |
What it is | A complication of an unrepaired congenital heart defect |
Most common cause | Ventricular septal defect (hole between the lower chambers) |
What happens | Lung blood vessels stiffen and narrow; pressure rises; oxygen levels fall |
Is the damage reversible? | No, the damage to lung vessels is permanent |
Main treatment | Medicines; repair of the hole is not recommended once it develops |
Follow-up | At least a yearly checkup with a congenital heart specialist |
Pregnancy | Very high risk; avoiding pregnancy is considered critical |
What is Eisenmenger syndrome?
Eisenmenger syndrome (pronounced I-sun-meng-ur) is a long-term complication of a congenital heart defect, which is a heart problem present at birth. It most often develops when a hole between two chambers of the heart is never repaired.
The hole sends extra blood into the lungs' arteries. Over time, this extra flow makes the blood vessels in the lungs stiffen and narrow. Pressure then rises in the lung arteries, and the walls of the right lower heart chamber, called the right ventricle, become thick.
The damage to the lung blood vessels is permanent. Because the condition is life-threatening, early diagnosis and repair of congenital heart defects usually prevents it from developing in the first place. [1] [2]
Symptoms
Symptoms of Eisenmenger syndrome can include the following.
Symptom | What it looks like |
Blue or gray skin | Color changes that may be harder or easier to see depending on skin color |
Chest pain or tightness | Discomfort or pressure in the chest |
Coughing up blood | Blood in the sputum when coughing |
Dizziness or fainting | Feeling lightheaded or losing consciousness |
Easily tiring | Shortness of breath during activity |
Headaches | Recurring head pain |
Clubbing | Large, rounded fingernails or toenails |
Numbness or tingling | Sensations in the fingers or toes |
Breathlessness at rest | Shortness of breath even without activity |
Irregular heartbeats | Skipped or racing heartbeats |
When to see a doctor
See a healthcare professional if you have any of these symptoms, even if you have never been diagnosed with a heart condition. Get emergency medical help for symptoms such as shortness of breath or chest pain.
Causes
Eisenmenger syndrome is usually caused by an unrepaired hole between the main blood vessels or chambers of the heart. This hole is called a shunt, and it is a congenital heart defect, meaning it is present at birth. The following defects can lead to the syndrome. [2]
Heart defect | Description |
Ventricular septal defect | The most common cause; a hole in the wall of tissue between the bottom heart chambers |
Atrioventricular canal defect | A large hole in the center of the heart where the walls between the upper and lower chambers meet, sometimes with valves that do not work as they should |
Atrial septal defect | A hole in the wall of tissue between the two upper heart chambers |
Patent ductus arteriosus | An opening between the artery that carries oxygen-poor blood to the lungs and the body's main artery |
In any of these conditions, blood flows in a way it usually does not. Pressure rises in the pulmonary artery, and over time the increased pressure damages the smaller blood vessels in the lungs. The damaged vessel walls make it hard for the heart to pump blood to the lungs.
The pressure also rises on the side of the heart that carries oxygen-poor blood, sometimes called blue blood. That blood passes through the hole, and oxygen-rich and oxygen-poor blood begin to mix. This mixing causes low blood oxygen levels throughout the body.
Risk factors
A family history of congenital heart defects increases the risk of similar heart problems in a baby. If you have been diagnosed with Eisenmenger syndrome, talk with your healthcare professional about screening other family members for congenital heart defects.
Complications
Eisenmenger syndrome is a life-threatening condition. How well someone does depends on the specific cause and whether other medical conditions are present. Possible complications include the following.
Complication | What happens |
Low blood oxygen levels | Changed blood flow sends less oxygen to the body's tissues and organs, and levels worsen without quick treatment |
Arrhythmias | Thickened heart walls and low oxygen may cause irregular heartbeats; some raise the risk of blood clots, heart attack, or stroke |
Sudden cardiac arrest | Sudden loss of heart activity that can quickly lead to death without immediate treatment |
Heart failure | Increased pressure weakens the heart muscle, making pumping harder |
Bleeding in the lungs | Life-threatening bleeding in the lungs and airways; bleeding can also occur elsewhere in the body |
Stroke | A blood clot traveling from the right to the left side of the heart may block a vessel in the brain |
Kidney disease | Low blood oxygen levels may impair the kidneys |
Gout | Increased risk of a type of arthritis causing sudden, severe pain and swelling, usually in the big toe |
Heart infection | Higher risk of endocarditis, an infection of the heart |
Pregnancy risks | High risk of death for both the pregnant person and the baby |
Diagnosis
A healthcare professional diagnoses Eisenmenger syndrome with a physical exam and questions about symptoms and medical history, supported by several tests.
Test | What it shows |
Blood tests | A complete blood count may show a high red blood cell count; other tests check the kidneys, liver, and iron levels |
Electrocardiogram (ECG or EKG) | The heart's electrical activity and how fast or slow it beats |
Chest X-ray | The condition of the heart and lungs |
Echocardiogram | Detailed moving images of the heart, its valves, and blood flow using sound waves |
CT scan of the lungs | Detailed images of the lungs and lung arteries, sometimes with contrast dye |
MRI of the lungs | Detailed images of the blood vessels in the lungs |
Cardiac catheterization | A thin tube guided to the heart measures pressures in the vessels and chambers, shows blood flow, and reveals the size of any hole |
Walking test | How the body reacts to mild exercise |
Treatment
The goals of treatment are to manage symptoms, improve quality of life, and prevent complications. Care usually moves to a cardiologist, a doctor trained in heart diseases, and it helps to find one experienced in treating congenital heart defects. Regular checkups, at least once a year, are an important part of treatment.
Medicines
Medicines are the main treatment. They cannot cure Eisenmenger syndrome, but they can improve quality of life.
Medicine | Purpose | Important notes |
Anti-arrhythmics | Control the heart rhythm and prevent irregular heartbeats | Taken as directed by the care team |
Iron supplements | Correct a low iron level | Never start without talking with the healthcare team |
Aspirin or blood thinners | Reduce clot risk after a stroke, blood clot, or some irregular heartbeats | Increase bleeding risk; never take unless the team says to |
Bosentan (Tracleer) | Helps send more blood to the lungs when pulmonary arterial hypertension is present | Requires regular blood tests because it can damage the liver; it can also cause serious birth defects, so pregnancy tests and reliable birth control are required |
Sildenafil and tadalafil | Open the blood vessels in the lungs so blood flows more easily | Used for high pressure in the pulmonary arteries |
Antibiotics | Prevent endocarditis before some dental and medical procedures | Only recommended in specific situations |
Surgeries and procedures
Once Eisenmenger syndrome has developed, healthcare professionals do not recommend surgery to repair the hole in the heart.
If red blood cell counts are too high and cause symptoms such as headaches or trouble seeing or concentrating, blood withdrawal (phlebotomy) may be needed. It should never be done routinely, only after consulting a congenital heart disease expert, and fluids are given through a vein during treatment to replace what is lost.
If other treatments do not work, some people may need a heart or lung transplant. People who need treatment should get care at a medical center with professionals experienced in congenital heart diseases.
Lifestyle and home remedies
Daily habits can meaningfully improve quality of life for someone living with Eisenmenger syndrome.
Drink plenty of fluids. Ask the healthcare team how much fluid is needed each day. More may be needed when sick, in a heated room, or on an airplane.
Ask whether exercise is OK. Really hard exercise or sports are not advised, but less intense physical activity may be possible. The healthcare professional can advise on what type of activity is best.
Avoid high altitudes. Low oxygen levels at altitude are dangerous. The American College of Cardiology and the American Heart Association advise avoiding exposure to altitudes of 5,000 feet (1,524 meters) or more. Ask the healthcare team for guidance before airplane trips or high-altitude travel.
Avoid situations that greatly lower blood pressure. Hot tubs, saunas, and long hot baths or showers can cause fainting and other complications. Prolonged straining, such as lifting heavy objects, should also be avoided.
Use medicines and supplements with care. Many over-the-counter and prescription medicines can affect blood pressure, increase bleeding risk, or affect kidney function. Talk with the healthcare team before taking anything new.
Get vaccinated. A flu shot every year and pneumococcal (pneumonia) vaccination as recommended by your healthcare team help prevent respiratory infections.
Do not smoke or use tobacco. Staying away from secondhand smoke matters too, because smoking increases the risk of complications.
Birth control and pregnancy
Pregnancy poses serious health risks, and can be life-threatening, for both the pregnant person and the baby. Healthcare professionals consider it critical to avoid pregnancy entirely if you have Eisenmenger syndrome.
Effective contraceptive options include vasectomy, an intrauterine device (IUD), a hormonal implant such as etonogestrel, and tubal ligation, although tubal ligation involves surgery and is less often recommended.
Birth control pills containing estrogen are not recommended because estrogen raises the risk of blood clots that could block an artery to the heart, brain, or lungs. Barrier methods alone, such as condoms or diaphragms, are not recommended because of the risk of failure.
Coping and support
Joining a support group can help. Many people find comfort and encouragement in talking with others who have been through similar situations. Ask the healthcare team about local groups.
Children with a congenital heart defect may face emotional and physical concerns affecting home and school life, and a long recovery from treatment may lead to developmental delays. The healthcare team can advise on support, including support groups or a therapist or psychologist.
Preparing for your appointment
Because Eisenmenger syndrome is complicated, coming prepared helps. Before the appointment, note any previous heart treatments, ask about pre-appointment restrictions, and write down symptoms with when they started. Bring a list of all medicines, vitamins, and supplements with dosages, along with family history details such as congenital heart defects, pulmonary hypertension, lung or heart disease, stroke, high blood pressure, or diabetes. Taking someone along can help remember the information shared.
Good questions to ask include what else could cause the symptoms, which tests are needed, what the treatment is, what side effects to expect, whether exercise should change, how often follow-ups are needed, and whether an experienced specialist can be recommended. The care team will likely ask when symptoms started, whether they come and go, how severe they are, and what improves or worsens them.
Conclusion
Eisenmenger syndrome is serious, but it is manageable with the right specialist care and daily habits. If you or someone you love has a congenital heart defect that was never repaired, start a conversation with a healthcare professional, even without symptoms.
Next steps: schedule a checkup with a cardiologist experienced in congenital heart disease, ask about screening for family members, and follow the daily care guidance above.
Related reading on Rinnit
Frequently asked questions
What is the most common cause of Eisenmenger syndrome?
The most common cause is an unrepaired ventricular septal defect, a hole in the wall of tissue between the two lower chambers of the heart. Other causes include an atrioventricular canal defect, an atrial septal defect, and patent ductus arteriosus.
Can Eisenmenger syndrome be cured?
No. Once the syndrome has developed, the damage to the lung blood vessels is permanent and medicines cannot cure it. Medicines are the main treatment and can manage symptoms, improve quality of life, and help prevent complications.
Is surgery recommended to fix the hole in the heart?
No. Once Eisenmenger syndrome has developed, healthcare professionals do not recommend surgery to repair the hole in the heart. Instead, treatment focuses on medicines, monitoring, and in some cases blood withdrawal or heart or lung transplant.
How often should someone with Eisenmenger syndrome see a doctor?
Regular checkups at least once a year are an important part of treatment, and care should be managed by a cardiologist experienced in congenital heart defects at a center familiar with these conditions.
Is pregnancy safe with Eisenmenger syndrome?
No. Pregnancy poses a high risk of death for both the pregnant person and the baby and is considered critical to avoid. Effective options such as an IUD, hormonal implant, or vasectomy are recommended, while estrogen-containing pills and barrier methods alone are not.
This article is for general education and is not a substitute for professional medical advice, diagnosis, or treatment. Always consult a qualified healthcare professional about your individual situation.
References
American Heart Association. The Impact of Congenital Heart Disease. American Heart Association. Last Reviewed August 25, 2026. https://www.heart.org/en/health-topics/congenital-heart-defects/the-impact-of-congenital-heart-defects
National Heart, Lung, and Blood Institute. What Are Congenital Heart Defects? NHLBI, NIH. Last updated March 24, 2022. https://www.nhlbi.nih.gov/health/congenital-heart-defects
Arvanitaki A, Giannakoulas G, Baumgartner H, Lammers AE. Eisenmenger syndrome: diagnosis, prognosis and clinical management. Heart. 2020;106(21):1638-1645. PMID 32690623. https://doi.org/10.1136/heartjnl-2020-316665
Chaix MA, Gatzoulis MA, Diller GP, Khairy P, Oechslin EN. Eisenmenger Syndrome: A Multisystem Disorder-Do Not Destabilize the Balanced but Fragile Physiology. Can J Cardiol. 2019;35(12):1664-1674. PMID 31813503. https://doi.org/10.1016/j.cjca.2019.10.002
Barradas-Pires A, Constantine A, Dimopoulos K. Preventing disease progression in Eisenmenger syndrome. Expert Rev Cardiovasc Ther. 2021;19(6):501-518. PMID 33853494. https://doi.org/10.1080/14779072.2021.1917995
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