Double Switch in Congenitally Corrected Transposition of the Great Arteries With Incomplete Atrioventricular Septal Defect, Ebsteinoid Tricuspid Valve, Ventricular Outflow Tract Obstructions, and Multiple VSDs

World J Pediatr Congenit Heart Surg. 2022 Jul;13(4):527-529. doi: 10.1177/21501351221085544. Epub 2022 Mar 11.


We describe the management of a 2 month-old male (BSA 0.27 m2) with congenitally corrected transposition of the great arteries (CCTGA), left and right ventricular outflow tract obstruction, incomplete atrioventricular (AV) canal defect, Ebsteinoid left AV valve, and multiple VSDs. In short, the patient presented with respiratory failure, metabolic acidosis with worsening AV valve regurgitation, and acute decompensation from pulmonary overcirculation. We performed a complex double switch operation described below. Despite the unique anatomical complexities, the patient's successful course demonstrates the importance of multidisciplinary discourse, preoperative imaging and planning, and intraoperative decision making from patient selection to anatomic repair.

MeSH terms

  • Congenitally Corrected Transposition of the Great Arteries
  • Heart Septal Defects
  • Heart Septal Defects, Ventricular
  • Humans
  • Infant
  • Male
  • Transposition of Great Vessels* / complications
  • Transposition of Great Vessels* / diagnostic imaging
  • Transposition of Great Vessels* / surgery
  • Tricuspid Valve / diagnostic imaging
  • Tricuspid Valve / surgery
  • Ventricular Outflow Obstruction* / diagnostic imaging
  • Ventricular Outflow Obstruction* / etiology
  • Ventricular Outflow Obstruction* / surgery

Supplementary concepts

  • Atrioventricular Septal Defect
  • Partial atrioventricular canal