Ebstein’s Anomaly

Abstract

EBSTEIN’S ANOMALY IS A RARE congenital heart defect characterized by displacement of the tricuspid valve leaflets into the right ventricle.1The defect was first described by Wilhelm Ebstein in 1866.2This anomaly of the tricuspid valve causes the right atrium to thin and become enlarged, resulting in a wide range of clinical presentations.3Clinical presentation depends on the severity of the pathologic findings, which vary considerably from patient to patient. Some infants may present with cyanosis, respiratory distress, heart failure, and even death, whereas others may not present with mild symptoms until adolescence or adulthood.