Combined Norwood and Modified Left-Sided Starnes Procedures for Congenitally Corrected Transposition of the Great Arteries

The combination of congenitally corrected transposition of the great arteries (ccTGA), Ebstein anomaly of the systemic atrioventricular valve, and critical systemic ventricular outflow tract obstruction is extremely rare. Management of a neonate with these lesions in cardiogenic shock is quite challenging. Tricuspid valve exclusion has been increasingly and successfully used to manage neonatal Ebstein anomaly patients. We extended this idea of tricuspid valve exclusion (modified Starnes) combined with a Norwood Stage 1 operation with a modified systemic to pulmonary artery shunt to successfully manage a neonate with ccTGA, severe systemic atrioventricular valve regurgitation, and critical ventricular outflow tract obstruction.