A rare cause of profound cyanosis after Kawashima modification of bidirectional cavopulmonary anastomosis

The expected level of systemic arterial saturation may not be present after bidirectional superior cavopulmonary anastomosis in children with complex congenital cardiac anomalies. We present a case of persistent severe cyanosis in a patient with azygos continuation of the inferior vena cava after bidirectional superior cavopulmonary anastomosis (Kawashima procedure) due to an intrahepatic venovenous malformation. Subsequent transcatheter deployment of two Rashkind double umbrella devices into the malformation reduced the shunt and markedly improved systemic arterial saturation.