Clinical and Imaging Predictors of Poor Prognosis in Ebstein Anomaly

Ebstein anomaly is a rare congenital heart condition characterized by the partial or complete absence of tricuspid valve delamination, a variable degree of “atrialization” of the right ventricle, and a redundant anterior leaflet with fenestrations. Tricuspid valve repair or replacement is often necessary to address tricuspid valve regurgitation, yet the timing remains controversial. Early intervention risks multiple reoperations, whereas disease progression may cancel the benefits of a delayed intervention. Clinical, biological, imaging, and electrocardiographic markers of poor prognosis may help determine when to intervene. Transthoracic echocardiography and cardiac magnetic resonance imaging are imaging modalities of choice to detect prognostic markers in the context of severe tricuspid regurgitation. This review summarizes established and emerging prognostic factors that may help to individualize the timing and planning of surgery for patients with Ebstein anomaly.