A case of bilateral absent pulmonary arteries

Herein, we present the case of a 17-month-old boy with asplenia, bilaterally absent pulmonary arteries, and bilateral patent ductus arteriosus, who underwent a successful Fontan operation. The central pulmonary artery was created using a pericardial roll, which was initially oversized due to the elevated pressure from the systemic-to-pulmonary shunt. The size of the roll was reduced through the process of pressure reduction by bidirectional Glenn and Fontan operations. This case provides an example of blood source-associated size transition of the pericardial roll used in the pulmonary position. Recognizing this phenomenon is vital for the successful management of this patient group.