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:
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:
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.
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