Bi-Ventricular Repair of Critically Ill Neonates With Ebstein's Anomaly – Keeping it Simple

Abstract

Surgical management of critically ill neonates with Ebstein anomaly (EA) remains challenging with most published hospital and 1-year mortalities approximating 30%-40% irrespective of the surgical or palliative strategy. We have adopted an initial bi-ventricular repair strategy for most symptomatic neonates and young infants since 1994 and sought to identify whether our simplified tricuspid valve repair will be associated with improved outcome by a wider audience of experienced surgeons. The essential principles of the repair include (i) monocusp tricuspid valve repair based on the anterior leaflet, (ii) fenestrated ASD closure), (iii) repair of all associated cardiac defects, and (iv) reduction right atrioplasty. The technique of tricuspid valve repair depicted in the following figures was associated with a 100% 1-year survival for the most recent 6 years (n = 9).