Description

Eisenmenger (I-sol-meng-ur) syndrome, is a long-term complication of an unrepaired condition of the heart that are present at birth is called a congenital defect of the heart. The Eisenmenger syndrome is life-threatening.

In the Eisenmenger syndrome, is not irregular blood flow in the heart and the lungs. This causes the blood vessels in the lungs become stiff and narrow. It raises the blood pressure in the pulmonary arteries. The Eisenmenger syndrome causes a permanent to the blood vessels in the lungs.

The early diagnosis and the repair of congenital heart defects in general prevents the Eisenmenger syndrome. If you do not develop, treatment consists of regular health examinations and medications to improve the symptoms.

Symptoms

The symptoms of Eisenmenger syndrome include:

  • Blue or gray skin. Depending on the color of the skin, these changes may be more difficult or more easy to see.
  • Chest pain or tightness.
  • Coughing up blood.
  • Dizziness or fainting.
  • Easily fatigue, and shortness of breath with activity.
  • Headaches.
  • Large, rounded nails of the hands or feet, it is called the night club.
  • Numbness or tingling in the fingers or toes of the feet.
  • Shortness of breath at rest.
  • Omit or heart palpitations.

When to see a doctor

If you have any of the symptoms of Eisenmenger syndrome, consult your health care professional. Make an appointment, even if you have never been diagnosed with a heart disease.

Get emergency medical help for symptoms such as shortness of breath or chest pain.

Causes

The Eisenmenger syndrome is usually caused by a repair the hole between the major blood vessels or chambers of the heart. The hole is called a derivation. A shunt is a problem of the heart that are present at birth, which means that it is a congenital defect of the heart.

Congenital heart defects that can cause Eisenmenger syndrome include:

  • The Ventricular septal defect. This is the most common cause of Eisenmenger syndrome. There is a hole in the wall of tissue between the bottom chambers of the heart.
  • Atrioventricular canal defect. This is a great hole in the center of the heart. The hole is where the walls between the upper and lower chambers chambers meet. Some of the valves of the heart may also not work as it should.
  • Atrial septal defect. This is a hole in the wall of tissue between the two upper chambers of the heart.
  • Patent ductus arteriosus. This is an opening between the artery that carries oxygen-poor blood to the lungs and the body's main artery.

In any of these conditions of the heart, the blood flows in a way that is not normally the case. As a result, the increased pressure in the pulmonary artery. Over time, the increased pressure damages the small blood vessels in the lungs. The damaged walls of the blood vessels make it difficult for the heart to pump blood to the lungs.

In the Eisenmenger syndrome, the blood pressure is elevated in the side of the heart is oxygen-poor blood, also called blue blood. The blue of the blood passes through the hole in the heart or the blood vessels. Oxygen-rich and oxygen-poor blood mixture now. This causes low levels of oxygen in the blood.

Risk factors

A family history of congenital defects of the heart, increases the risk of similar problems of the heart in an infant. If you have been diagnosed with Eisenmenger syndrome, talk with your healthcare provider about the tests of other members of the family for congenital heart defects.

Complications

The Eisenmenger syndrome is a life-threatening condition. How well the person with Eisenmenger syndrome depends on the specific cause and if there are other medical conditions.

The complications of the Eisenmenger syndrome may include:

  • Low levels of oxygen in the blood. The change in the flow of blood through the heart sends less oxygen to the body's tissues and organs. Without quick treatment, the levels of oxygen in the worse.
  • Irregular heartbeat, also called arrhythmias. The Eisenmenger syndrome makes the heart walls to get bigger and thicker. Also causes a drop in oxygen levels. These changes can lead to an irregular heartbeat. Some irregular heartbeats increase the risk of blood clots that can cause heart attacks or strokes.
  • The sudden cardiac arrest. This is the sudden loss of heart function due to an irregular heart rhythm. If not treated immediately, sudden cardiac arrest, can quickly lead to death. Survival is possible with the fasting, proper medical care.
  • Heart failure. The increased pressure on the heart can cause the heart muscle is weakened. It becomes more difficult for the heart to pump blood.
  • Bleeding in the lungs. The Eisenmenger syndrome can cause life-threatening bleeding in the lungs and airways. The bleeding may also occur in other parts of the body.
  • Stroke. If a blood clot that travels from the right side to the left side of the heart, the blood clot can block a blood vessel in the brain. A blood clot in the brain can cause a stroke.
  • Kidney disease. The low oxygen levels in the blood, which can lead to problems with the kidneys.
  • Gout. The Eisenmenger syndrome may increase the risk of a type of arthritis called gout. Gout causes sudden, severe attacks of pain and swelling in one or more joints, usually the big toe of the foot.
  • Infection in the heart. People with Eisenmenger syndrome have a higher risk of a heart infection called endocarditis.
  • Risk in pregnancy. During pregnancy, the heart and lungs have to work harder to support the growing baby. Because of this, the pregnancy with Eisenmenger syndrome, involves a high risk of death in both the pregnant person and the baby. If you have Eisenmenger syndrome, talk with your healthcare provider about the risks of pregnancy.

Diagnosis

To diagnose the syndrome Eisenmenger, your healthcare provider will examine you and ask questions about your symptoms and medical history.

Tests

The tests to diagnose the Eisenmenger syndrome may include:

  • Blood tests. A complete blood count is often done. The number of red blood cells in the blood may be high in the Eisenmenger syndrome. Blood tests are also done to see how well your kidneys and liver are working. Another blood test to check iron levels.
  • Electrocardiogram (ECG or EKG). This quick and painless test that measures the electrical activity of the heart. During an ECG , adhesive patches with sensors attached to the chest and, at times, the arms or the legs. Connect the cables from the sensors to a machine, which displays or prints the results. An EKG can show how fast or slow the heart is beating.
  • X-ray of the chest. An x-ray of the chest shows the condition of the heart and the lungs.
  • The echocardiogram. The sound waves create detailed images of the heart in motion. An echocardiogram shows the flow of blood through the heart and the heart valves.
  • The computed tomography (CT) of the lungs. This type of ct scan uses X-rays to create detailed pictures of the lungs and the arteries of the lung. CT scan images provide more-detailed information than plain x-rays. Dye, called contrast, may be given through a vein (IV) for this test. The dye helps the blood vessels to appear more clearly in the images.
  • Magnetic resonance imaging (MRI) of the lungs. This test uses magnetic fields and radio waves to create detailed images of blood vessels in the lungs.
  • The cardiac catheterization. This test shows how the blood flows through the arteries of the heart. A doctor places a thin, flexible tube called a catheter into a blood vessel, usually in the groin or the wrist, and the guide for the heart. Contrast medium flows through the tube to the arteries. The dye helps the arteries to show up more clearly on X-ray images and video. The pressure in the blood vessels and chambers of the heart can be measured during this test. Cardiac catheterization can also show the size of the holes in the heart.
  • Walking test. You can ask to walk for several minutes to see how your body reacts to mild exercise.

Treatment

The objectives of Eisenmenger syndrome treatment are:

  • Manage the symptoms.
  • Improve the quality of life.
  • To prevent complications.

If you have Eisenmenger syndrome, which are usually sent to a doctor trained in diseases of the heart, called a cardiologist. It is useful to find a cardiologist with experience in the treatment of people who have congenital heart defects. Regular check-ups at least once a year — are an important part in the treatment of Eisenmenger syndrome.

Drugs

Medications are the main treatment for the Eisenmenger syndrome. The drugs can't cure the Eisenmenger syndrome, but they can help to improve the quality of life.

Medications used to treat the Eisenmenger syndrome include:

  • Medicines to control heart rhythm. These drugs are called anti-arrhythmics. Help to control the heart rhythm and prevent irregular heartbeats.
  • Iron supplements. Your health care professional may suggest that these if your iron level is very low. Do not start taking iron supplements without talking with your health care team.
  • Aspirin or anticoagulants. If you have had a stroke, blood clots, or some types of irregular heartbeats, you may need to take an aspirin or a blood thinner such as warfarin (Jantoven). These medicines may increase the risk of bleeding. Never take them unless your health care team tells you to.
  • Bosentan (Tracleer). This medicine is used if you have pulmonary arterial hypertension. Help send more blood to the lungs. If you take this medication, you need blood tests because the drug may cause liver damage.
  • Sildenafil and tadalafil. Sildenafil (Revatio, Viagra) and tadalafil (Cialis, Adcirca, other) are sometimes used to treat high blood pressure in the pulmonary arteries caused by Eisenmenger syndrome. These medicines open up the blood vessels in the lungs to allow blood to flow more easily.
  • Antibiotics. Some dental and medical procedures can stop germs into the bloodstream. Some people need to take antibiotics before surgery or a dental procedure to prevent a heart infection called endocarditis. Preventive antibiotics are recommended only in specific situations. Talk with your healthcare provider to know if they are right for you.

Surgeries or other procedures

Health professionals do not recommend the surgery to repair the hole in the heart once the Eisenmenger syndrome has been developed.

Surgeries or procedures that may be done to treat the symptoms or complications of Eisenmenger include:

  • The blood of withdrawal, also called phlebotomy. If your red blood cell count is too high and is causing symptoms such as headaches or difficulty to see or focus, it is possible that need this treatment. Phlebotomy should not be done routinely, and should only be done after talking with congenital heart disease expert. Fluids should be administered through a vein (IV) during the treatment to help replace the fluids lost.
  • Heart or lung transplant. If other treatments for Eisenmenger syndrome do not work, some people may need surgery to replace the heart or the lungs.

If you need treatment for Eisenmenger syndrome, get medical attention at a medical center with the health professionals that have experience in congenital heart disease.

Lifestyle and home remedies

If you have Eisenmenger syndrome, these tips can help you improve your quality of life.

  • Drink plenty of fluids. Ask your health care team how much fluid you need each day. You may need to drink more fluids when they are sick or when you're in a heated room or on an airplane.
  • Ask if the exercise and sports are good. If you have Eisenmenger syndrome, you should not make very difficult the exercise or sport. But you can be able to make less intense physical activities. Ask your health care professional what type of physical activity is the best for you.
  • Avoid high altitudes. Due to the low oxygen levels at high altitudes, people with Eisenmenger syndrome should not spend a lot of time at high altitudes. The American College of Cardiology and the American Heart Association says to avoid exposure to a height of 5,000 feet (1,524 m) or more above sea level. If you plan to take a trip on a plane or traveling to high altitudes, ask your health professional for specific guidance.
  • Avoid situations that can significantly reduce the blood pressure. For example, do not sit in a hot tub or in the sauna, or taking long hot baths or showers. Such activities can lower blood pressure and can cause fainting or other complications. If you have Eisenmenger syndrome, also avoid activities that cause prolonged effort, such as lifting heavy objects or weights.
  • The use of medications and supplements with care. Many supplements and medicines, including those bought without a prescription, can affect blood pressure. Some may increase the risk of bleeding or affect kidney function in people who have Eisenmenger syndrome. Talk with your health care team before taking any new supplements or medications.
  • Get vaccinated against the flu. If you have Eisenmenger syndrome, it is especially important to prevent respiratory infections like the flu. Get vaccinated against the flu every year and a pneumonia vaccine every five years.
  • Do not smoke or use tobacco. Also stay away from second hand smoke. Smoking can increase the risk of Eisenmenger syndrome complications.

Birth control and pregnancy

If you have Eisenmenger syndrome, pregnancy poses serious risks to the health and can be life-threatening — for you and for the baby. Health professionals say that it is critical that the pregnancy should be avoided if you have the Eisenmenger syndrome.

Effective methods of contraception include vasectomy, intrauterine device (IUD) or contraceptive hormone implant, such as etonogestrel (Nexplanon). The ligation of the fallopian tubes, also called tubal ligation, is a very effective form of contraception. But it is less often recommended due to the risks posed by having even a minor surgery.

Birth control pills that contain estrogen are not recommended for those who have Eisenmenger syndrome. Estrogen increases the risk of developing blood clots that could block an artery of the heart, brain, or lungs. The use of a barrier method, like a condom or a diaphragm, it is not recommended due to the risk of failure.

Coping and support

If you have Eisenmenger syndrome, you may find that joining a support group is of great help. Some people find that talking with others who have gone through similar situations brings comfort and encouragement. Ask your health care team if there are any local support groups.

Children with a congenital defect of the heart or Eisenmenger syndrome can have emotional and physical issues that may affect the home and the school of life. For example, a long recovery time of a medical treatment can lead to development delays. Ask your health care team about the ways that you can help your child. This may include support groups, or a visit to a therapist or psychologist.

Preparing for your appointment

The Eisenmenger syndrome is a complicated condition. There's often A lot to discuss at a medical appointment. So it's a good idea to be prepared. Here's some information to help you prepare for your appointment and know what to expect from your health care professional.

What you can do

  • Take note of the previous heart of the treatments. Include medicines, surgery, or other treatments for heart problems.
  • Be aware of any pre-appointment restrictions. When you make the appointment, ask if there is something that you need to do in advance, such as filling out forms or not to eat or drink anything for a while. For example, you or your child may be asked not to eat or drink anything for a couple of hours before a test image.
  • Write down your symptoms, including any that may seem unrelated to the Eisenmenger syndrome. Try to remember when they started. To be specific, such as days, weeks, and months.
  • Write important personal information, including a family history of congenital heart defects, pulmonary hypertension, lung disease, heart disease, stroke, high blood pressure or diabetes, and any major stresses or recent life changes.
  • Make a list of all the medicines, vitamins, and supplements you are taking. Include the dose. Also keep in mind any of the medications or supplements that you or your child has recently stopped taking.
  • Bring someone along, if possible. Sometimes it can be difficult to remember all the information provided to you during an appointment. Someone who goes with you may remember something that you missed or forgot.
  • Write questions to ask their health professional.

His time with the health care professional may be limited. Have a list of questions prepared can help you make the most of their time together. A list of questions from most important to least important in case time runs out. For the Eisenmenger syndrome, some basic questions to ask the health professionals include:

  • What are other possible causes of the symptoms?
  • What kind of proof do you need?
  • What is the treatment?
  • What are the side effects of treatment?
  • How to recommend changes to exercise or sports?
  • What is the frequency of follow-up visits necessary?
  • Can you recommend a specialist who has experience in the treatment of Eisenmenger syndrome?
  • Are there brochures or other printed material that I can take my house? What sites do you recommend?

Do not hesitate to ask other questions.

What to expect from your doctor

Your health care team will probably ask a lot of questions. Be prepared to answer them you can save your time to go through the information that you want to spend more time. Your health care team may ask:

  • Have you ever been diagnosed with a heart disease or high blood pressure in the lungs? If so, what treatments did you have for your condition?
  • When did the symptoms begin?
  • They are always the symptoms, or the symptoms come and go?
  • How bad are the symptoms?
  • What, if anything, seems to improve your symptoms?
  • Does anything make the symptoms worse?
Symptoms and treatment of Eisenmenger syndrome