A patient with congenital heart disease and a left-to-right shunt may develop the Eisenmenger Syndrome (or Complex) as s/he becomes older.


Diagnostic features of the Eisenmenger Syndrome:

(1) congenital heart disease initially with a left-to-right shunt

(2) development of secondary pulmonary hypertension due to increased vascular resistance within the lung

(3) switch from a left-to-right to right-to-left shunt, with blood bypassing the lungs

(4) hypoxemia

(5) erythrocytosis which may progress to hyperviscosity syndrome


Clinical findings and complications:

(1) hypoxemia, manifested as dyspnea on exertion, easy fatigability, cyanosis, and/or syncope

(2) clinical findings of pulmonary hypertension, with right atrial and right ventricular enlargement

(3) congestive heart failure

(4) cardiac arrhythmias

(5) hemoptysis

(6) stroke

(7) thromboembolic events

(8) cholelithiasis

(9) renal dysfunction

(10) hypertrophic osteoarthropathy ("clubbing")

(11) gout

(12) hyperviscosity syndrome, which may present as headache, dizziness, visual disturbances or subtle neurologic abnormalities


The magnitude of the right-to-left shunt may increase whenever there is an decrease in the systemic vascular resistance, causing increased cyanosis. This may occur with:

(1) hot weather

(2) exercise

(3) fever

(4) systemic infection


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