Eisenmenger Syndrome

1 What is Eisenmenger Syndrome?

Eisenmenger syndrome is a complication caused by serious congenital defects in the heart. Eisenmenger syndrome happens when blood low in oxygen circulates throughout the body. It is caused by a shunt or hole between the left side and the right side of the heart, causing abnormal blood circulation.

At first, the shunt causes blood from the left side (which has higher pressure) of the heart to travel to the heart’s right side that has lower pressure. This increases blood flowing to the lungs that have smaller and narrower arteries. This results in hypertension of right side of the heart and gradual destruction of blood circulation of the lungs.

Over time, the blood pressure in the right side of the heart becomes so high that it now adds blood to the heart’s left side. Blood from the right side of the heart is low in oxygen, and since it mixes with blood at the heart’s left side, is pumped throughout the body resulting in insufficient oxygen levels to the organs and tissues. The low oxygen levels in the bloodstream due to heart defects and pulmonary hypertension causes Eisenmenger syndrome and its complications.

Because it develops over time, Eisenmenger syndrome tends to manifest in adolescents and young adults. These patients unknowingly have a congenital heart defect at birth. If left untreated, Eisenmenger syndrome results to life-threatening complications or even death.

2 Symptoms

Eisenmenger syndrome may cause several symptoms, many of which are related to too low oxygen levels in the blood. However, note that patients may also experience other symptoms related to problems in blood circulation in the lungs.

Here are the symptoms of Eisenmenger syndrome:

  • Large, rounded fingernails and toe nails (clubbing)
  • Bluish or grayish color of the skin and lips (cyanosis)
  • Marked shortness of breath that stays even at rest
  • Palpitations
  • Tightness or pain in the chest
  • Dizziness
  • Fainting
  • Headaches
  • Numbing or tingling of fingers and toes
  • Noticeable abdominal swelling
  • Coughing up blood or frothy sputum tinged with blood

You need to see a doctor quickly if you have these symptoms. Even if you were not diagnosed to have heart defects, cyanosis with shortness of breath are symptoms that there is something wrong in the heart, which needs immediate medical attention.

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3 Causes

Eisenmenger is caused by serious congenital defects in the heart.

Eisenmenger syndrome happens when low-oxygen blood makes its way to the arteries that supply blood throughout the body, resulting to very low oxygenation of the organs and tissues. To fully understand the condition, you need to know the basics of the function of the normal heart and circulatory system.

The heart has four chambers; two atria that hold blood before pumping, and two ventricles that pumps blood.

The heart is roughly divided into two sections, the left side and the right side, each with one atrium and a ventricle. The left side of the heart receives oxygenated blood from the lungs and pumps it to arteries supplying the whole body, so naturally it has significantly higher blood pressures. The right side of the heart receives low-oxygen (deoxygenated) blood from the veins of the whole body and pumps it to the lungs to be enriched with oxygen, and it operates at lower blood pressures. Obviously, the blood from left and the right side of the heart must never mix.

Eisenmenger syndrome originates from heart defects that allows deoxygenated blood and oxygenated blood to blend together.

These defects are congenital or develop before birth. Here are those heart defects:

  • Ventricular septal defect –this is caused by a hole between the left and right ventricles, which are the pumping chambers of the heart. This is the most common.
  • Atrial septal defect – this is the hole between the left and right upper chambers of the heart that holds blood before it transfers into the ventricles.
  • Patent ductus arteroisus – This is a hole or pathway between the pulmonary artery, which carries deoxygenated blood to the lungs, and aorta that carries oxygenated blood.
  • Atrioventricular septal defect - this happens when there is a large hole between the atrium and ventricles of the heart. This causes the heart to become malformed, and the valves separating the chambers may not work properly at all.

At the start, these heart defects forms a shunt that allow blood from the heart’s left side to go to the right side. This happens because the heart’s left side has higher pressure than the right side. The right side supplies blood to the lungs through the pulmonary artery, so additional blood cause elevation in blood pressure in the lungs. The problem is that the pulmonary artery and arteries in the lungs have thinner walls making them incapable of handling higher pressures. This results to hypertension of the lungs (pulmonary hypertension) that cause damage to the blood vessels.

Over time, usually at adolescent or early adult age, pulmonary hypertension becomes so severe that the high blood pressure causes blood from heart’s right side to now transfer to the heart’s left side through the shunt. This causes deoxygenated blood to be pumped throughout the bloodstream, resulting very low oxygen levels in the body that characterize Eisenmenger syndrome. Low oxygen levels cause cyanosis, clubbing of nails and shortness of breath even at rest. The brain is also affected by low oxygen, resulting in dizziness, headaches and fainting spells.

Eisenmenger syndrome is a consequence of untreated congenital heart defects. A baby with a heart defect must receive prompt surgery to correct the defects and avoid Eisenmenger syndrome.

4 Making a Diagnosis

Diagnosing Eisenmenger syndrome requires physical examinations, studying your medical and family history and diagnostic tests.

Your doctor may ask you to have the following diagnostic tests:

  • Electrocardiogram (ECG) measures the electrical signals of the heart using electrodes attached to your skin, so it requires little preparation. ECG is used to check for heart defects and problems in heart functioning. You might only have to shave the area of the skin in which the electrodes will be attached.
  • Chest X-rays, which is used by the doctor to visualize your heart and lungs to check for enlarged arteries.
  • Echocardiogram is the ultrasound of the heart and produces detailed images of the structure of your heart to check for defects.
  • Computerized tomography (CT) scan, which uses a big machine that uses several computer-directed X-rays scans to produce very detailed images. CT scans are used by doctors to take cross-section images of your lungs.
  • Magnetic resonance imaging (MRI) uses a powerful magnetic field to visualize organs of your body in good detail. Your doctor may use MRI to visualize the blood vessels in your lungs. MRI requires you to discard all metal items like jewelry, pins, buttons or piercings.
  • Walking stress test measures your heart (or sometimes lung function) as you are subjected to mild exercise.
  • Cardiac catheterization is an invasive procedure wherein a thin flexible tube called catheter is inserted in your groin and guided to your heart. From the catheter, the doctor may measure the amount of blood and blood pressures inside your heart’s chambers, check the size of heart defect, or inject a dye to make your heart stand out clearly to X-rays.

If you will undergo cardiac catheterization, choose a cardiologist with expertise in diagnosing and treating Eisenmenger syndrome.

5 Treatment

Treatment for Eisenmenger syndrome is focused on controlling and management of symptoms. This approach helps improve your overall health and quality of life, and most importantly, prevent the development of serious complications.

There is no medical procedure to reverse the effects of Eisenmenger syndrome. Since the condition is serious, it is important that you receive care from a cardiologist experienced in treating Eisenmenger syndrome.

Surgery to correct heart defects is not routinely performed to treat Eisenmenger syndrome. The condition causes many health problems makes surgery very risky to the patient.

Routine checkups and observation is part of the treatment. This means you have to visit your cardiologist at least once a year, and more frequently if you have ongoing health problems. In a typical appointment, you may undergo a physical examination, blood tests and reviewing of your symptoms or complaints to determine the condition of your heart and your body.

Medications

The doctor will prescribe you medications to relieve symptoms of Eisenmenger syndrome. You will be continuously monitored to check for side effects and see if the medication works for you.

Here are the medications:

  • Antiarrhythmics are medicines that correct arrhythmia or very fast and irregular heartbeats, which often happens in Eisenmenger syndrome.
  • Iron supplements help increase iron levels in the body if it is too low. Iron helps your blood carry more oxygen. However, do not take iron supplements without first asking your doctor.
  • Blood thinners like aspirin help prevent the formation of clots in your bloodstream. The doctor may prescribe aspirin if you have had a stroke, arrhythmia or formed blood clots.
  • Endothelin receptor antagonists like bosentan (Tracleer) prevent narrowing of your blood vessels that may help improve your energy levels and reduce hypertension of blood vessels in the lungs. The side effect is potential harm to the liver, so you need to have a monthly liver test when taking the drug.
  • Antihypertensive medications like sildenafil (Revatio, Viagra) and taladafil (Cialis, Adcirca) may help relieve hypertension of blood vessels in the lungs. These drugs may cause stomach irritation, dizziness and vision problems as a side effect.
  • Antibiotics may be prescribed in case of infections or before having dental or medical procedures to kill bacteria that can cause problems in heart valves.

Only take medicines prescribed by your doctor. Over-the-counter medicines may not be safe for you, so talk to your doctor first before taking them.

Phlebotomy

Some cases of Eisenmenger syndrome cause the body to produce too much red blood cells to compensate for the lack of enough oxygen in the body. This can result in thickening of the blood, which is difficult for the heart to pump. If you have this problem, the doctor may recommend and perform phlebotomy or bloodletting to reduce red blood cell counts. You will be given intravenous fluids to replace lost fluids in the body.

Heart-lung transplantation

In case your heart and lungs sustained much damage, your doctor may recommend either lung transplant or heart-lung transplant. These are complex major surgical procedure involving transplantation of a lung or heart and two lungs to replace diseases organs. Transplantation is an option if other treatments fail, and if you have matched to a donor organ.

Birth control

It is important for female patients with Eisenmenger syndrome not to get pregnant. Pregnancy can cause serious risk to the mother and the developing baby due to problems in the circulatory system and oxygen levels in the body.

Birth control pills are not recommended because of increased risk of blood clot formation. Barrier devices like condoms and diaphragm may fail. Though tubal ligation only involves minor surgery, it can still remain risky to patients.

For Eisenmenger syndrome, the doctor may recommend you to have a permanent birth control method. The most preferred method is Essure, which involves inserting a metal coil to block the fallopian tubes permanently. This procedure is done through the vagina, so there is no need to do incisions.

6 Lifestyle and Coping

Lifestyle modifications are necessary in order to cope with Eisenmenger syndrome.

Eisenmenger syndrome is associated with several health problems especially if it becomes severe. Patients without very severe congenital heart defects tend to benefit from early and appropriate treatment.

It is possible to live a normal, active life even with Eisenmenger syndrome as long as you stick to these important rules:

  • Stick to exercise restrictions recommended by your doctor. You can still engage in less intense physical activities.
  • Do not go to high-altitude regions, which are places around 5000 feet (1,524 meters) or more above sea level; this includes travelling by air. Places in high-altitude tend to have thinner air and lesser oxygen, which could provoke symptoms.
  • Avoid situations that could cause your blood pressure to rise, such as long baths or showers, hot tubs, and saunas. You should also avoid straining activities like lifting heavy objects and weights. Such activities can result in rapid hypertension that can cause fainting or even death.
  • Be cautious in taking medications and supplements. These may cause increased risk of bleeding, affect kidney function or cause drastic changes in blood pressure. Always consult your doctor first before taking any medicine, supplement or vitamins – including over-the-counter ones.
  • Avoid tobacco and cigarette smoke. Tobacco damages your lungs, which already have problems getting oxygen in the air, and clogs your arteries.

Eisenmenger syndrome is associated with several health concerns.

The condition can be life changing for you or your child and can cause the following issues:

  • Emotional difficulties due to drastic changes in lifestyle and daily activities. You may be stressed thinking that the condition will worsen. This can be evident in parents whose child is diagnosed with the condition.
  • Children with Eisenmenger syndrome may feel insecure due to restrictions in activity and long recovery times after procedures or surgeries. This may result in emotional and developmental difficulties, which can get worse once the child reaches school age.

You can handle these issues better by establishing good emotional and social support. Discuss these with your doctor and ask for ways to cope. You can also ask the doctor to refer you to a therapist or psychologist, or support groups.

7 Risks and Complications

Leaving congenital heart conditions untreated is the risk factor for Eisenmenger syndrome, and treatment can reduce or prevent the condition.

Eisenmenger syndrome may cause serious complications if left untreated. Here are the complications:

  • Worsened cyanosis or too low oxygen levels in the bloodstream. This can drastically lower oxygen perfusion to the organs and further reduce your capacity for physical activities.
  • Stroke, or blockage of an artery supplying blood to the brain. Stroke is mostly caused by a blood clot, which can form due to arrhythmias, erythrocytosis, or pooling of blood in the legs.
  • Kidney conditions. The kidneys can sustain damage from too low oxygen levels in the blood, resulting in kidney failure. The kidneys may also fail to remove uric acid, increasing your risk of having gout.
  • Erythrocytosis or too high numbers of red blood cells. Low oxygen levels caused by Eisenmenger syndrome can cause the body to create too many red blood cells. Erthyrocytosis thickens the blood, which is harder for your heart to pump, resulting in reduced blood flow to organs and increased tendency to form blood clots.
  • Arrhythmia or too rapid heartbeats. Eisenmenger syndrome affects the heart function, and combined with too low oxygen levels, can cause the heart to beat more rapidly and out of rhythm. Arrhythmias are undesirable because rapid heartbeat can cause the formation of blood clots that can block an artery and cause stroke, heart attack or pulmonary thrombosis.
  • Sudden cardiac arrest, or unexpected and sudden cessation of heart activity. This stops heartbeat and halts blood flow to the brain and vital organs. Death can result in minutes unless addressed immediately.
  • Heart failure or too insufficient heart beat to supply blood needed by the body. Complications of Eisenmenger syndrome may weaken the muscles of the heart that adversely affects its pumping efficiency.
  • Expectoration of blood or hemoptysis. Hemoptysis is caused by seepage of blood in the lungs. Increased blood pressure in the lungs can cause the tiny blood vessels in there to burst.
  • Pregnancy risks. Women with Eisenmenger syndrome must not get pregnant. Eisenmenger syndrome results to low blood supply and low oxygenation, so pregnancy poses grave danger to the mother and the developing fetus.
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