Hypoplastic left heart syndrome, interrupted inferior vena cava, biliary atresia

A neonate was diagnosed with hypoplastic left heart syndrome, heterotaxy syndrome, and interrupted inferior vena cava soon after birth. At 6 days old she underwent the Norwood procedure using Sano modification. Postoperatively she had persistent direct hyperbilirubinemia and was diagnosed with biliary atresia for which she underwent a Kasai procedure at 29 days old. At 10 months she underwent the Kawashima procedure. She is now 20 months old and has been thriving without any jaundice. This case report illustrates that even in the presence of major multiple congenital anomalies, staged reconstruction for hypoplastic left heart syndrome can be successfully performed.