Eisenmengers Syndrome - Symptom, Causes, Treatment

Eisenmenger's syndrome is a progressive heart condition. The signs and symptoms of Eisenmenger's syndrome often do not occur until childhood or early adulthood. Diagnosis of Eisenmenger's syndrome is made with an echocardiogram. The syndrome may also occur in complex cyanotic heart defects such as a heart with a single ventricle. The syndrome can develop with different types of congenital heart defects. Symptoms occur because of low oxygen levels in the blood and because the right heart may enlarge and ultimately fail as it pumps at a much higher pressure than it was built for. Symptoms include breathlessness on effort, fatigue, palpitations, chest pain, fainting and coughing up blood. The pulmonary hypertension together with right to left shunting of blood is known as the Eisenmenger's Syndrome. Heart rhythm disturbances such as atrial fibrillation are also common and can cause a decrease in the overall performance of the heart. People with Eisenmenger's Syndrome are usually born with a large hole in the heart. The most common situation where this occurs is when there is a hole,(defect),between the two pumping chambers, (ventricles), and is called a ventricular septal defect.

Eisenmenger's syndrome primarily affects adolescents and adults with certain congenital heart defects that are repaired late (after the first year of life) or that are never repaired. Right heart failure, (inability of the heart muscle to maintain adequate forward blood flow to meet the body's demand), and often tricuspid valve regurgitation appear in the later stages of the syndrome. In Eisenmenger's syndrome, there are two major abnormalities present that increase the risk of stroke. First, the right to left shunt can allow air bubbles, bacteria and small blood clots that normally would be 'filtered out', of the blood stream in the lungs direct access to the arterial circulation of the brain. Secondly, the very high haemoglobin levels associated with too many RBC's may promote clot formation. Treatment is generally supportive, but heart and lung transplantation may be an option when symptoms are severe. Endocarditis prophylaxis is recommended.

Causes of Eisenmengers syndrome

The main causes of Eisenmengers syndrome:

• Congenital heart defects.

Signs and symptoms of Eisenmengers syndrome

Signs and symptoms often experienced with Eisenmengers syndrome are:

• Shortness of breath at rest.
• Chest pain.
• Blurred vision.
• Stroke.
• Brain abscesses.
• Fatigue.
• Dizziness or syncope.

Treatment of Eisenmengers syndrome

Some most common treatmet of Eisenmengers Syndrome:

• Treatment is generally supportive, but heart and lung transplantation may be an option when symptoms are severe.
• Endocarditis prophylaxis is recommended.
• Supplemental oxygen may be used during sleep periods or while resting. Use of oxygen may progress to continuous use for symptom relief.
• Phlebotomy (removal of blood) may be recommended when erythrocytosis causes hyperviscosity of the blood.
• Lung transplantation or heart-lung transplantation may be performed when other treatments are no longer effective.

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