After total correction of tetralogy of Fallot, pulmonary atresia and major aorto-pulmonary collateral arteries, a 31-year-old man developed life-threatening pulmonary hypertension and reperfusion pulmonary edema, leading to pulmonary hemorrhage, right heart failure and hypoxia. Because of difficulty in weaning from cardiopulmonary bypass and in controlling hemorrhage from pulmonary arteries, we applied percutaneous cardiopulmonary support (PCPS) for 281 hours with strategy of delayed sternal closure (17 days) and a large quantity of transfusion. We also applied lung protective ventilatory strategy for reperfusion pulmonary edema (high PEEP, limited peak inspiratory pressure and recruitment maneuver). As the result, he was discharged alive without any major complications. We report our postoperative managements, in terms of 1) lung protective ventilatory strategy, 2) surgical control of pulmonary blood flow, and 3) evaluation of the cardiopulmonary function during PCPS for early weaning from PCPS.