Eisenmenger syndrome can cause dangerous heart problems. It mostly affects teenagers and adults with certain heart defects. However, it can also affect children, although it is very rare.
Early diagnosis and repair of congenital heart defects usually prevent Eisenmenger syndrome. If it does develop, treatment involves regular medical visits and medications to improve symptoms.
Therefore, check out the review about what Eisenmenger syndrome is, its symptoms, causes, and how to treat it.
Getting to Know Eisenmenger Syndrome
Quoting fromMayo Clinic, Eisenmenger syndrome is a long-term complication of an unrepaired heart problem that is present at birth (congenital heart defect). This heart complication can be life-threatening if not treated promptly.
Eisenmenger syndrome is a rare condition that affects the heart and lungs. The arteries that carry blood from the heart to the lungs become narrowed, making the pressure of the blood against the artery walls (blood pressure) too high. The heart has to work harder to pump blood to the lungs. This causes lung damage.
The type of high blood pressure experienced by affected individuals is called pulmonary arterial hypertension, which affects the blood vessels in the lungs and the right ventricle of the heart. Typically, people with Eisenmenger syndrome are born with a heart defect (congenital heart defect) that is not corrected by surgery or other intervention at an early age.
A normal heart has four chambers. The two upper chambers, known as the atria, are separated from each other by a fibrous partition, known as the atrial septum. The two lower chambers are known as the ventricles and are separated from each other by the ventricular septum.
Valves connect the atria (left and right) to their respective ventricles. These valves allow blood to be pumped through the chambers. Blood flows from the right ventricle through the pulmonary artery to the lungs where it receives oxygen.
Blood returns to the heart through the pulmonary veins and enters the left ventricle. The left ventricle pumps the now oxygenated blood into the body’s main artery (aorta). The aorta carries blood throughout the body.
Children with Eisenmenger syndrome often have a ventricular septal defect or “hole in the heart” between the left and right pumping chambers of the heart. This causes significant shunting of blood from the left side of the heart to the right at birth, which progresses to pulmonary vascular disease.
Once pulmonary vascular disease develops, the heart defect can no longer be repaired. Eventually blood flow through the defect (shunt) may become bidirectional, leading to cyanosis (reduced oxygen concentration in the blood).
Symptoms of Eisenmenger Syndrome
The specific symptoms of Eisenmenger syndrome vary greatly from person to person. Although the heart defect is present at birth, Eisenmenger syndrome with cyanosis often develops during puberty.
This syndrome may also develop earlier or later depending on the location and severity of the congenital heart defect.
Symptoms and complications result from the combined effects of heart defects, reduced oxygen in the blood and high pressure in the lungs. The most prominent symptom is called cyanosis , which is a bluish discoloration of the skin and mucous membranes.
Children affected by Eisenmenger syndrome develop cyanosis, especially of the lips, fingers and toes, which is more prominent during physical effort.
Patients typically have an increased number of blood cells (red blood cells or erythrocytes) that carry oxygen to the body (i.e. erythrocytosis), compensating for the inadequate oxygen supply to the tissues.
The following are the most common symptoms of Eisenmenger syndrome:
- Blue or grayish skin due to lack of oxygen in the blood (cyanosis)
- Shortness of breath with activity
- Shortness of breath at rest
- Feeling tired
- Chest pain or chest tightness
- Skipping or racing with the heartbeat
- Headache
- Dizziness or fainting (syncope)
- Numbness or tingling in the fingers and toes, or both
- Blurred vision
- Changes in fingers and toesclubbing)
- Swelling
- Liver enlargement
Reason
Quoting fromMedline Plus, 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 pumping chambers – the left and right ventricles – of the heart (ventricular septal defect).
The hole allows blood that has picked up oxygen from the lungs to flow back into the lungs, instead of out to the rest of the body.
Other heart defects that can cause Eisenmenger syndrome include:
- Atrioventricular canal defect
- Atrial septal defect
- Cyanotic heart disease
- Patent ductus arteriosus
- arterial trunk
Over the years, increased blood flow can damage the small blood vessels in the lungs. This causes high blood pressure in the lungs. As a result, blood flows backward through the hole between the two pumping chambers. This allows oxygen-poor blood to travel throughout the body.
Eisenmenger syndrome may begin to develop before a child reaches puberty. However, it can also develop in young adulthood, and can progress throughout young adulthood.
Frequency of Occurrence
Eisenmenger syndrome is rare. It occurs in 1% to 6% of adults born with a heart defect.
In addition, Eisenmenger syndrome usually develops in children and young adults who have an uncorrected ventricular septal defect (VSD). A VSD is a congenital heart defect in which there is a hole in the wall (septum) between the two lower pumping chambers of the heart (the left and right ventricles).
Children with Down syndrome are also at higher risk for congenital heart defects and Eisenmenger syndrome. Between 25% and 50% of adults with Eisenmenger syndrome have Down syndrome .
Risk Factors
A family history of congenital heart defects increases the risk of similar heart problems in a baby. If your child has been diagnosed with Eisenmenger syndrome, talk to your doctor about screening other family members for congenital heart defects.
Diagnosis
Doctors and healthcare providers usually look for signs of Eisenmenger syndrome during a physical exam. They may suggest the following tests to confirm the diagnosis:
1. Blood Test
A complete blood count is often done. The red blood cell count may be high in Eisenmenger syndrome. Blood tests are also done to check kidney and liver function and iron levels.
2. Electrocardiogram (ECG)
This quick, painless test measures the electrical activity of the heart. During an EKG, sensors (electrodes) are attached to the chest and sometimes to the arms or legs. Wires connect the sensors to a machine, which displays or prints the results. An EKG can show how fast or slow the heart is beating.
3. Chest X-ray
A chest X-ray helps show the condition of the heart and lungs.
4. Echocardiography
An ultrasound of the heart is called echocardiography. This test uses sound waves to create detailed images of the heart in motion. Echocardiography shows blood flow through the heart.
5. Computerized Tomography (CT) Scan
A CT scan uses X-rays to create cross-sectional images of certain parts of the body. This test may be done to get more details about the lungs and pulmonary arteries.
A dye (contrast) may be given through an IV for this test. The dye helps the blood vessels show up more clearly on the images. CT scan images provide more detailed information than regular X-rays.
6. Magnetic Resonance Imaging (MRI) Scan of the Lungs
This test uses magnetic fields and radio waves to create detailed images of the blood vessels in the lungs.
7. Cardiac Catheterization
A long, thin, flexible tube (catheter) is inserted into a blood vessel, usually in the groin or wrist, and guided to the heart. Dye flows through the catheter into the arteries.
The dye helps the arteries show up more clearly on X-ray images and videos. During this test, the provider can measure blood pressure in the blood vessels and chambers of the heart. Cardiac catheterization can also help show the size of the hole in the heart and blood flow in that area.
Handling
The goal of treatment for Eisenmenger syndrome is to control symptoms, improve quality of life, and prevent serious complications.
If your child is diagnosed with Eisenmenger syndrome, he or she will be referred to a heart specialist (cardiologist). Regular checkups, at least once a year, are an important part of treating Eisenmenger syndrome. It is helpful to find a cardiologist who has experience treating people with congenital heart defects.
Drugs
Medications are the main treatment for Eisenmenger syndrome. They cannot cure the condition, but they can help improve your little one’s quality of life. Parents also need to have regular health checks while taking medication. The doctor will also check for any changes in blood pressure, pulse, and fluid levels.
Medications for Eisenmenger syndrome include:
1. Heart Rhythm Medication
These drugs, called antiarrhythmics, help control heart rhythm and prevent irregular heartbeats.
2. Iron Supplements
Your healthcare provider may prescribe this if your iron levels are too low. Don’t start taking iron supplements without talking to your provider first.
3. Aspirin or Blood Thinners
If you’ve had a stroke, blood clot, or certain types of irregular heart rhythm, your healthcare provider may recommend aspirin or a blood thinner such as warfarin (Jantoven). These medications can increase your risk of bleeding.
Never take it unless your provider tells you to do so . Ask your provider if other pain relievers, such as ibuprofen (Advil, Motrin IB, others), are safe for you.
4. Medication for Pulmonary Arterial Hypertension
A medicine called bosentan (Tracer) can be used to increase blood supply to the lungs. If you are taking bosentan, you will need monthly liver tests because the medicine can damage the liver.
5. Sildenafil and Tadalafil
Sildenafil (Revatio, Viagra) and tadalafil (Cialis, Adcirca, others) are sometimes used to treat high blood pressure in the pulmonary arteries caused by Eisenmenger syndrome. These drugs open up blood vessels in the lungs so blood can flow more easily.
6. Antibiotics
Certain dental and medical procedures can allow bacteria to enter the bloodstream. Depending on your condition, you may need to take antibiotics before undergoing these procedures.
Antibiotics can help prevent heart infection (endocarditis). If you have any type of congenital heart disease, talk to your dentist and other care providers about your risk and whether you need preventive antibiotics.
Operation or Other Procedure
Health care providers do not recommend surgery to repair the hole (Shunt) in the heart after Eisenmenger syndrome develops.
Surgeries or procedures that may be performed to treat symptoms or complications of Eisenmenger’s include:
1. Blood Collection (Phlebotomy)
If your red blood cell count becomes too high and causes symptoms such as headaches, difficulty concentrating or vision problems, your provider may recommend this procedure.
Phlebotomy should not be performed routinely and should only be done after consultation with a congenital heart disease specialist. You will need to receive IV fluids while undergoing this procedure to help replace lost fluids.
2. Transplantation
Some people who have Eisenmenger syndrome may need surgery to replace their heart or lungs if other treatments don’t work.
If you need a procedure or surgery for Eisenmenger’s, consider getting care at a medical center with providers experienced in congenital heart disease.
Prevention
The only way to prevent Eisenmenger syndrome is to correct congenital heart defects as early as possible.
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That’s the information about Eisenmenger syndrome, hopefully it’s useful!
Republished with permission from theAsianParent Indonesia
