Eisenmenger's syndrome

(Victor Eisenmenger, 18641932, German physician) irreversible severe pulmonary arterial hypertension resulting from excessive pulmonary blood flow over a long period. Eisenmenger initially described a patient with a large ventricular septal defect who had developed cyanosis. This term is now used to describe the clinical syndrome and pathophysiology resulting from reversal of any left to right shunt. It can be produced by atrial septal defect, ventricular septal defect, patent ductus arteriosus, atrioventricular septal defect (atrioventricular canal), truncus arteriosus, aorticopulmonary window and other lesions. Plain radiography reveals prominently enlarged main and central pulmonary arteries frequently with discrepancy in size of central and peripheral pulmonary arteries (Fig.1). Typically, the cardiac size is normal or only slightly enlarged. The right-sided chambers may be enlarged. Doppler echocardiography can confirm the high pulmonary arterial pressure by interrogating the peak velocity of a tricuspid regurgitant jet employing the modified Bernoulli equation. Echocardiography and MRI demonstrate the pulmonary arterial and right-sided chamber enlargement and show the responsible cardiac or aortic congenital anomaly. MRI is very effective for demonstrating right ventricular hypertrophy and showing the flattening or reversal of curvature of the ventricular septum (Fig.2).

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