[Absent pulmonary valve syndrome: surgical approach for the worst group included in symptomatic neonates]

Absent pulmonary valve syndrome (APVS) has been classified to two groups. One is severely symptomatic infant group and the other no or slightly symptomatic. But we think that severely symptomatic group should be divided into the worst neonate group and the other. This worst group, that contains neonates who can not be weaned from the respirator after surgical intervention including corrective surgery because of severe bronchomalacia and/or peripheral bronchial stenosis, has already severe respiratory distress and needs ventilatory support while high pulmonary vascular resistance is maintained. Three patients of this group were operated on at 2, 13 and 2 days of age and there were two late hospital death. The last patient underwent ligation of main pulmonary artery (mPA) and left modified Blalock-Taussig shunt (MBTS) with phi 4 mm polytetrafluoroethylene graft and was extubated on the next day. The management of this group should aim at controlling the pulmonary regurgitation as early as possible to decrease the progressive airway obstruction and minimize pulmonary tissue damage. Ligation of mPA and MBTS can be performed without cardiopulmonary bypass and eliminates pulmonary regurgitation and controls the pulmonary blood flow less than the total correction. In the point of protecting bronchi and lung and reducing the risk of surgical intervention in critical neonatal period, ligation of mPA and MBTS can be safer and more effective operation for the worst APVS neonate than the total correction with insertion of artificial valve.