Successful management of critical neonatal Ebstein's anomaly with circular shunt

The prognosis of neonatal Ebstein's anomaly requiring early surgery is poor, especially with a circular shunt. We report a critically ill case managed with emergency two-stage Starnes surgery immediately after birth. We hypothesised that the three components of the Starnes procedure, right ventricle exclusion, atrial septal defect creation, and systemic-to-pulmonary shunt, could be organised and reconstructed in a consecutive two-stage strategy to control postoperative circulatory fluctuations.