A long-lasting complication of a congenital heart defect (an untreated heart condition that is present at birth) is called Eisenmenger syndrome. This condition is life-threatening and it causes irregular blood flow in the heart and lungs. As a result, the blood vessels in the lungs become stiff and narrow, which makes blood pressure increase in the arteries of the lungs (also called pulmonary hypertension). Furthermore, this syndrome causes permanent damage to the blood vessels in the lungs.
The only way to prevent this condition is early diagnosis and treatment of the congenital heart defect.
Symptoms
People with this condition usually experience the following symptoms. Examples include:
- Dizziness
- Coughing up blood
- Fainting
- Chest tightness or pain
- Gray or blue skin
- Breathing problems
- Headaches
- Clubbing (large and rounded toenails and fingernails)
- Numbness in the toes or fingers
- Fast heart rate
You should contact your healthcare professional right away if any of the previous symptoms occur.
Causes
Commonly, this syndrome occurs due to an unrepaired hole that happens between main blood vessels or heart chambers. The hole is called a shunt and this is a congenital heart defect. Check below for some examples of congenital heart defects that cause Eisenmenger syndrome:
- Ventricular septal defect – This is a condition that causes a hole between the bottom chambers of the heart and it is the most common cause of Eisenmenger syndrome.
- Atrioventricular canal defect – People with this congenital heart defect have a large hole in the heart center between the upper and lower chambers. In such cases, heart valves also may not work properly.
- Atrial septal defect – In such cases, the hole occurs between the upper chambers of the heart.
- Patent ductus arteriosus – People with this congenital heart defect have an opening between the artery that carries oxygen-poor blood to the lungs and the body’s primary artery.
However, any of the previous conditions make blood flow in an unusual way causing blood pressure in the pulmonary artery to increase. As a result, damage to the blood vessels in the lungs occurs, which makes it difficult for the heart to pump blood to the lungs.
Risk Factors
The risk of Eisenmenger syndrome increases if you have a family history of congenital heart defects. It is also advised to discuss with your healthcare professional about screening your family members for congenital heart defects if you were diagnosed with Eisenmenger syndrome.
What Are The Potential Complications of Eisenmenger Syndrome?
People with this syndrome may also experience some complications, especially if they do not get treatment. For example:
- Reduced oxygen levels in the blood often happen due to changes in blood flow through the heart. This is a medical emergency that requires quick treatment.
- Arrhythmias – This is a condition in which irregular heart rhythms occur. Sometimes, this complication may increase the risk of blood clots and cause myocardial infarctions or strokes.
- Sudden cardiac arrest – This is a medical emergency that is caused by sudden loss of heart activity. Without quick treatment, this complication is fatal.
- Heart failure – Weakness of the heart muscle may occur due to high pressure in the heart. Thus, it makes it difficult for the heart to pump blood.
- Bleeding in the lungs – This condition may cause dangerous bleeding in the lungs and airways, which may be fatal. However, bleeding may occur in other body parts too.
- Stroke – This complication happens when a blood clot goes through the bloodstream to the brain to block a blood vessel that supplies the brain with oxygen-rich blood.
- Kidney disease – Certain kidney problems can occur due to reduced oxygen levels in the blood.
- Gout – People with Eisenmenger syndrome are at higher risk of developing this arthritis type. This condition causes sudden pain and swelling either in one or more joints. However, it mostly negatively affects the big toe.
- Heart infection – At higher risk of developing endocarditis are people with this syndrome.
- Pregnancy risks – Generally, it is not recommended to become pregnant if you have Eisenmenger syndrome because it poses high risks of death for both the pregnant woman and the baby.
Diagnosis
Usually, doctors to determine whether you have this syndrome will ask you some questions about the symptoms and medical history and will perform some tests that will help to confirm the condition and rule out others that cause similar symptoms. For example:
- Blood tests – These tests are done to measure the red blood cell count, iron level, and organ function (such as kidney and liver).
- Electrocardiogram (EKG or ECG) – This is a quick test that measures the electrical activity of the heart and can show how the heart is beating.
- Chest X-ray – It checks for heart and lung conditions.
- Echocardiogram – This test uses sound waves to produce images of the heart in motion.
- CT (computerized tomography) scan – In such cases, this test is used to get detailed images of the lungs and their arteries.
- MRI (magnetic resonance imaging) scan – This test helps get detailed images of the blood vessels in the lungs.
- Cardiac catheterization – This is a specific test that shows how the blood flows through the heart arteries. It involves a small and flexible tube (catheter) into a blood vessel (often in the wrist or groin) and then guided to the heart.
Treatment
The treatment goal is to reduce the symptoms, improve your quality of life, and prevent complications. Check below the most common treatment options recommended by doctors:
Medicines
Medications are the primary treatment for people with Eisenmenger syndrome. While the following medicines cannot cure the condition, they can lessen the symptoms and improve your quality of life. For example:
- Anti-arrhythmic medications – These medicines are used to prevent irregular heartbeats.
- Iron supplements – Some people may need these supplements because Eisenmenger syndrome may cause reduced iron levels in the blood.
- Aspirin or Anticoagulants – People with blood clots, stroke, or irregular heartbeats usually need to administer a blood thinner (such as Warfarin) and Aspirin. However, do not take previous medicines without a doctor’s approval because it may lead to an increased risk of bleeding.
- Bosentan – This is an effective medicine for people with pulmonary hypertension. It works by sending more blood to the lungs. However, it may cause damage to the liver so, take it exactly as prescribed by your doctor.
- Erectile dysfunction (ED) or pulmonary hypertension medicines – These include Sildenafil and Tadalafil. These medications work by widening the blood vessels in the lungs making the blood flow more easily.
- Antibiotics – These medications are recommended if you have dental or certain medical procedures that allow the bacteria to enter the body. Antibiotics often are helpful to prevent a heart infection (such as endocarditis).
Surgery and Other Treatments
It is not recommended to perform surgeries or procedures to close the opening (hole) in the heart once the condition happens. Check below some procedures used to lessen the symptoms and prevent complications:
- Phlebotomy (blood withdrawal) – This procedure is used in patients who have increased red blood cell count that usually causes the following symptoms including visual problems, headaches, difficulty concentrating, and others.
- Heart or lung transplant – Physicians recommend this surgery when other treatment options do not work.
Frequently Asked Questions
What are the primary symptoms of Eisenmenger syndrome?
These include:
- Blurred vision
- Numbness in the toes and fingers
- Chest pain
- Breathing problems, especially during activity
- Cyanosis (gray or blue skin tone)
However, there are multiple health conditions that may cause these symptoms. That’s why it is important to visit a doctor for diagnosis and proper treatment. Do not take any medicines without a doctor’s approval because it may lead to unpleasant complications.
What are the potential complications of Eisenmenger syndrome?
People with this condition may experience some complications, especially if they do not get treatment. For example:
- Kidney problems (such as kidney disease or failure)
- Gout
- Gallstones
- Pregnancy complications
- Blood clots
- Brain problems (including stroke, bleeding in the brain, infections)
- Heart problems (such as sudden cardiac arrest, irregular heart rhythms, infections, heart failure, myocardial infarction, and others)
This article does not contain all possible complications of this syndrome. For more details, discuss it with your healthcare professional.
Can Eisenmenger syndrome be cured?
Unfortunately, there is no way to cure the condition but there are several treatment options to reduce the symptoms, prevent complications, and improve your quality of life. Sometimes, it is possible to prevent Eisenmenger syndrome may repairing the congenital heart defect as soon as possible. If you have additional questions, ask your healthcare provider.
