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Cyanotic condition in which patients with an initially intracardiac (or surgically created extracardiac) left-to-right shunt show shunt reversal (i.e., now right-to-left shunting) secondary to significantly increased pulmonary vascular resistance (PVR).

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Eisenmenger Disease.

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The Austrian physician Viktor E. Eisenmenger was the first to describe in 1897 severe pulmonary vascular disease in a 32-year-old man with cyanosis and dyspnea since infancy secondary to an unrestricted ventricular septal defect, who died of massive pulmonary hemorrhage.

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Approximately 8% of patients with congenital heart disease and 11% of those with left-to-right intracardiac shunting develop Eisenmenger reaction.

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Eisenmenger reaction per se is not inherited; it is an acquired complication of a congenital cardiac lesion. No sexual predilection has been reported.

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In congenital cardiac lesions with intracardiac shunting, blood initially shunts from the high-pressure systemic circulation to the low-pressure pulmonary circulation. If the defect is unrestricted and sustained, exposure of the pulmonary vascular bed to systemic arterial pressure initially results in increased pulmonary blood flow and pressure that over time triggers progressive adaptation processes in the microvasculature, including arteriolar intima proliferation, media hypertrophy, and finally capillary and/or arteriolar occlusion. Obliteration of pulmonary arterioles and capillaries may result from necrotizing arteritis. Decreased endothelium-dependent pulmonary arteriolar dilatation and increased pulmonary endothelin and plasma thromboxane B2 levels (both pulmonary vasoconstrictors) have been described; all of these factors ultimately lead to increased PVR. Once systemic and pulmonary vascular resistances and pressures approach each other, a process called shunt reversal occurs, where the blood now shunts bidirectionally or from the right (pulmonary) side to the left (systemic) side. Reduced pulmonary blood flow and right-to-left shunting explain the arterial hypoxemia. Endothelium-dependent pulmonary arteriolar relaxation is impaired, pulmonary endothelin production is increased, and plasma thromboxane B2 concentrations are elevated in patients with the Eisenmenger reaction, suggesting that endothelial dysfunction or platelet activation plays a causative role in this condition.

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Dyspnea, chest pain (right ventricular ischemia), and new cyanosis in the presence of congenital heart disease. Echocardiography or cardiac catheterization (Caveat: radiographic contrast material may cause systemic arterial vasodilatation) can be used to determine the degree of pulmonary hypertension and demonstrate and quantify the shunting.

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The term Eisenmenger reaction describes cyanosis secondary to severe pulmonary hypertension in the systemic range as a result of significantly increased PVR with reversed or bidirectional shunting resulting from a connection between systemic and pulmonary circulation (such as septal defects, atrioventricular canal, patent ductus arteriosus, or aortopulmonary windows). Obtain a history of cyanotic episodes, syncopes, fatigue, dyspnea, check for finger clubbing, plethora, and polycythemia, which may be associated with hyperviscosity. Exercise tolerance is significantly reduced because of the inability to increase pulmonary blood flow. Hemoptysis is a common finding and often caused by pulmonary infarction, rupture of a pulmonary artery aneurysm, or a thin-walled pulmonary arteriole. There is an increased risk of hyperuricemia (increased production and reduced renal clearance of urate), cholelithiasis, hypertrophic ...

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