Eisenmengers Syndrome

Eisenmengers syndrome is condition with severe pulmonary arterial hypertension due to a shunting of blood from the left side of the heart to the right side of the heart. Eisenmenger's syndrome was so named by Dr. Paul Wood after Dr. Victor Eisenmenger, who first described the condition in 1897 . People who have Eisenmenger's syndrome are usually born with a large hole in 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, (VSD). 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. Diagnosis of Eisenmenger's syndrome is made with an echocardiogram (ultrasound of the heart). Other tests may include:

• An electrocardiogram (ECG), which can suggest increased right heart pressure (right ventricular hypertrophy)
• An X-ray of the chest to look for heart and pulmonary artery enlargement.

Eisenmengers Syndrome Causes and Symptoms

Eisenmenger's syndrome develops when high pressures push the blue (low oxygen) blood in the right side circulation back into the red (oxygenated) left sided circulation, bypassing the lungs. A person with Eisenmenger's Syndrome is paradoxically subject to the possibility of both uncontrolled bleeding and random clots. The large number of red cells tends to overpower the platelets. Characteristics of Eisenmenger's syndrome summarized:

• persistent truncus arteriosus
• ventricular septal defect
• left-right ventricular shunt
• right ventricle hypertrophy

Symptoms include breathlessness on effort, fatigue, palpitations, chest pain, fainting and coughing up blood. 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. 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. Cyanosis, clubbing of the fingers and increased red blood cells, (erythrocytosis), are all signs of the body's response to decreased oxygen. The tricuspid valve, (valve between the right atrium and right ventricle), will start to leak blood backwards, (regurgitation), as the right ventricle enlarges and stretches apart the valve leaflets. This will add to the workload on the heart as it tries to maintain forward blood flow. Heart rhythm disturbances such as atrial fibrillation are also common and can cause a decrease in the overall performance of the heart. Heart rhythm disturbances should be evaluated and treated promptly. Cyanosis - is the term used for the blue tinge to the skin especially the lips, finger tips and toes that is present when there is a low level of oxygen in the circulating blood. In Eisenmenger's syndrome, cyanosis is usually progressive.

Treatment of Eisenmengers Syndrome

Observational studies and case stories indicate however that the effect of Bosentan or Sildenafil in patients with Eisenmengers syndrome may be as promising in these patients as in patients with primary pulmonary arterial hypertension.

• Treatment for people with Eisenmenger's Syndrome is aimed at controlling symptoms and preventing complications.
• Medication is the primary treatment option.
• Medication is used to control rhythm disturbances and optimize heart muscle contraction.
• Medication is also used to treat the symptoms of heart failure such as fluid build up in the legs, abdomen and lungs.
• Many medications used to treat the symptoms of Eisenmenger's Syndrome will affect blood pressure, fluid and electrolyte values, (sodium, potassium etc), and heart rate.
• These medications require close monitoring.
• A rapid change in blood pressure, fluid volume or pulse rate could cause more right to left shunting and decrease oxygen levels to a critical level.
• Anticoagulants are medications to prolong the time it takes for a blood clot to form, like Aspirin or Warfarin, and are often used in this condition.
• Heart and lung transplantation is an option for some people with Eisenmenger's Syndrome.

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