Surgical Management of Ebstein Malformation of the Tricuspid Valve

Arman Arghami, MD, Elizabeth H. Stephens, MD, PhD, Joseph A. Dearani, MD


Ebstein malformation (EM) is a myopathy of the right ventricle and is characterized by embryonic failure of delamination of the septal, inferior and anterior leaflets of the tricuspid valve (TV) resulting in adherence of the leaflets to the underlying right ventricular (RV) myocardium. The anatomic changes associated with Ebstein malformation create infinitely variable abnormal TV morphology with varying degrees of TV regurgitation. EM can present as a spectrum of disease from the critically ill newborn to an asymptomatic adult.

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Last updated: February 10, 2022