The Surgical Management of Ebstein Anomaly

Among all congenital heart disease, Ebstein anomaly is the lesion with the widest spectrum of presentation. It can present as early as the neonatal period where immediate intervention is often needed to the adult with no discernable symptoms. It is also one where overall outcome greatly relates to age at presentation. Generally, presentation in the neonatal period is associated with poor outcomes while presentation beyond the neonatal period has excellent outcomes with low operative mortality. It is thus apparent that understanding every aspect of the anatomy and physiology of Ebstein anomaly and how it manifests clinically is paramount to successful treatment. The purpose of this review is to outline the current concepts and strategies in the surgical management of Ebstein anomaly, and to discuss the current expert consensus on how best to tackle this congenital heart disease at different ages of diagnosis.