Tag Archives: Endocardial cushion defect

8/4 Morning Report w/ Moyukh and Dr. Meadows: Chest Pain in a patient with Eisenmenger’s 

A middle aged man w/ Downs syndrome, unrepaired complete AV canal defect now w/ Eisenmenger’s syndrome c/b polycythemia presents with one episode of chest pain. Given the congenital abnormalities + polycythemia, the patient was at high risk for PE (embolism or in-situ), coronary artery compression (due to PA enlargement or elevated PA pressures) or coronary emboli – he was ruled out for these as well as ACS and ultimately discharged with a ziopatch to rule out arrhythmia.

*Teaching points

1) If a patient with an unrepaired endocardial cushion defect survives and develops Eisenmenger’s (shunt reversal to R -> L), they may be cyanotic but are at least protected from sudden worsening of R sided pressures and right heart failure. They’ve reached an equilibrium of pressures and are no longer candidates for repair.

2) Patients w/ endocardial cushion defect + shunt reversal have polycythemia/erythrocytosis (to compensate for chronic hypoxia) and thickened pulmonary artery walls. 

Because of this pathophysiology, they are at risk for:

embolic complications

-pulmonary embolism

-in-situ pulmonary thrombus

-hemoptysis

-coronary artery emboli

-stroke

-infected cerebral aneurysm/abscess

other complications

-coronary compression (due to enlarged or very high pressure PA)

-atrial arrhythmias 

-ventricular arrhythmias*

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