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 osteoarthropathy, cerebral
abscesses, and thromboembolic events (cerebrovascular insults). Renal
function may be decreased, with evidence of glomerulopathy (hematuria,
proteinuria, increased serum creatinine levels) reported in these patients.
Syncope, increased right-sided filling pressures, and severe hypoxemia
(i.e., systemic SpO2 <85%) are associated with a poor prognosis.
Sudden death as a result of arrhythmia and hypoxemia, but also pulmonary
artery rupture, has been reported. Death as a result of Eisenmenger reaction
usually occurs in the third decade of life. Patients with sleep apnea in
association with congenital heart disease may demonstrate accelerated
development of the disorder (e.g., patients with Trisomy 21).