Eisenmenger syndrome usually develops before puberty but may develop in adolescence and early adulthood.
Eisenmenger syndrome occurs in patients with big inborn cardiac or surgically created extracardiac left-to-right shunts. These shunts initially induce increased pulmonary blood flowing. People who have Eisenmenger's syndrome are normally born with a big hole in the eye. The most common situation is a hole between the two pumping chambers, called a ventricular septal defect. Usually, Eisenmenger syndrome develops while individuals with heart defects are still children, but it may occur in adolescence or young adulthood. A number of congenital heart defects can cause Eisenmenger's syndrome, including atrial septal defects, ventricular septal defects, patent ductus arteriosus, and more complex types of acyanotic heart disease.
Eisenmenger’s syndrome primarily affects adolescents and adults with sure inborn eye defects that are repaired later or that are never repaired. Because the pressures within the left position of the eye are usually greater than those within the correct position of the eye, a beginning between the left and correct position of the eye will induce blood to flood from the left position of the eye into the correct position. The symptoms of Eisenmenger’s syndrome may resemble other medical conditions or heart problems. A cardiac catheterization is an invasive procedure that gives very detailed information about the structures inside the heart. Eisenmenger syndrome specifically refers to the combination of pulmonary hypertension and right-to-left shunting of the blood within the heart. Eisenmenger's syndrome in rare instances may also develop with an atrial septal defect.
Symptoms related specifically to pulmonary hypertension result from the inability to increase pulmonary blood flow in response to physiological stress. A person with Eisenmenger's syndrome is paradoxically subject to the possibility of both uncontrolled bleeding due to damaged capillaries and high pressure, and random clots due to hyperviscosity and stasis of blood. The syndrome affects both males and females. Eventually, due to increased resistance, pulmonary pressures may increase sufficiently to cause a reversal of blood flow, so blood begins to travel from the right side of the heart to the left side, and the body is supplied with deoxygenated blood, leading to cyanosis and resultant organ damage. Eisenmenger syndrome is first suspected when an individual begins to show symptoms of pulmonary hypertension.
In early childhood, surgical intervention can repair the heart defect, preventing most of the pathogenesis of Eisenmenger's syndrome. Avoid very hot or humid conditions, which may exacerbate vasodilation, causing syncope and increased right-to-left shunting. If treatment has not taken place, heart-lung transplant is required to fully treat the syndrome. If this option is not available, treatment is mostly palliative, using anticoagulants, pulmonary vasodilators such as bosentan, antibiotic prophylaxis to prevent endocarditis, phlebotomy to treat polycythemia, and maintaining proper fluid balance. It is important to eat a nutritious diet and avoid alcohol and salt. Overexertion and smoking also should be avoided. Some patients might benefit from nocturnal supplementation, although it is most useful as a bridge to heart-lung transplant.
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