Objectives: To describe the various surgical options, perioperative management strategies, and outcomes for this complex lesion.
Data source: MEDLINE and PubMed.
Conclusions: L-transposition of the great arteries is a relatively rare defect, comprising less than 1% of all congenital heart defects. There is considerable heterogeneity in clinical presentation depending on the presence of associated defects. Surgery for this rare and complex defect has evolved from "physiologic" repair in which the right ventricle remains in the systemic position to the "anatomic" repair, which restores the left ventricle as the systemic ventricle. There has been a great interest in performing the anatomic repair or "double switch" operation, a rather complex operation that presents unique challenges in the postoperative period.