Relief of pulmonary venous obstruction for asplenia syndrome associated with total anomalous pulmonary venous connection in neonates and infants

: Five patients of asplenia syndrome with pulmonary venous obstruction underwent TAPVC repair in the period of neonates (4 patients) and infant (1 patient). They also underwent associated procedures to reconstruct or adjust pulmonary blood flow (systemic-pulmonary shunts in 2, bilateral PDA banding in 1, pulmonary artery banding in 1) in accordance with individual anatomy of the pulmonary arteries. In four of them, they required subsequent surgical procedures for reduction of pulmonary blood flow because of intractable heart failure due to increase in pulmonary blood flow mostly at the early postoperative period. The increasing pulmonary blood flow was successfully controlled by early reduction procedures (1 patient: extrathoracically adjustable PA banding, 1 patient: tightening of shunt graft) in two patients. However, one patient died in the early postoperative period because of moribund preoperative condition. The remaining one patient underwent PA banding three month after the first operation, but died in late period due to heart failure. These results suggest that the adjustment of pulmonary blood flow is critically important for management of the patients of asplenia syndrome with TAPVC after the operation, and early decision of PA band re-adjustment (or other procedures to decrease pulmonary blood flow) is mandatory to improve the results.