EBSTEIN ANOMALY AND MALFORMATION

Course overview

EBSTEIN ANOMALY IS A RARE CONGENITAL ABNORMALITY INVOLVING THE TRICUSPID VALVE AND THE RIGHT VENTRICLE. EBSTEIN ANOMALY OCCURS WHEN AN APICAL DISPLACEMENT OF THE TRICUSPID VALVE WITH TETHERING OF LEAFLET ATTACHMENTS. BOTH THE TRICUSPID VALVE AND THE RIGHT VENTRICLE ARE DYSPLASTIC, WITH IT FREQUENTLY DESCRIBED AS "ATRIALIZATION OF THE RIGHT VENTRICLE." EBSTEIN ANOMALY OFTEN RESULTS IN PATHOLOGICAL TRICUSPID REGURGITATION, RIGHT VENTRICULAR FAILURE, AND ARRHYTHMIAS. COMMON CLINICAL MANIFESTATIONS INCLUDE EXERTIONAL DYSPNEA, PALPITATIONS, AND CYANOSIS. THIS ACTIVITY REVIEWS THE PATHOPHYSIOLOGY AND PRESENTATION OF EBSTEIN ANOMALY AND HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM IN ITS MANAGEMENT.