Surgical Decision Making in Neonatal Ebstein’s Anomaly: An Algorithmic Approach Based on 48 Consecutive Neonates

Objective: There is currently no consensus of opinion regarding the optimal surgical management of Ebstein’s anomaly (EA) in neonates and young infants. Reported early mortality rates range from 25% to 100%. In this study, we present an algorithm for choosing the best management option for neonates with EA based on analysis of our experience. Patients and Methods: From 1994 to June, 2011, 48 neonates with a diagnosis of EA were managed by the same surgical team. Of these, two died before intervention; the remaining 46 either were managed medically initially (n = 20) or underwent surgical intervention during the neonatal period (n = 26) or early infancy (n = 9). Results: The mean weight was 3.6 ± 1.7 kg (1.9-8.6) and mean follow-up time was 6.3 ± 4.5 years (0.2-16). Of the 20 patients initially managed medically, 11 remain well without intervention and nine required complete repair in infancy, with 100% survival. Of the 26 neonatal operations, 23 (88%) were complete biventricular repairs, 1 Starnes’ pallia...