Ebstein’s anomaly is a rare congenital cardiac malformation that may present in the neonatal period with life-threatening physiologic derangement, especially when it is associated with circular shunt. Urgent surgical intervention is critical for survival because of hemodynamic compromise. We review our experience with surgical management of neonates with Ebstein’s anomaly associated with circular shunt and review the literature for similar reported cases. The underlying pathophysiology and different options of treatment are discussed.
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