Kawashima procedure after staged unifocalizations in asplenia with major aortopulmonary collateral arteries

We report a Kawashima procedure (total cavopulmonary shunt) successfully carried out for asplenia syndrome, pulmonary atresia, and major aortopulmonary collateral arteries. At the age of 8, the patient underwent staged bilateral unifocalizations using confluent central pulmonary arteries concomitant with bilateral modified Blalock-Taussig shunts. As the result of an interrupted inferior vena cava with azygous continuation, the patient required a Kawashima procedure with augmentation of the central pulmonary arteries for definitive palliation 1 year later. Cyanosis, respiratory distress, and ventricular function improved.