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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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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 ...