[Surgical strategy for pulmonary atresia with ventricular septal defect associated with severely hypoplastic or absent central pulmonary artery].

In patients of pulmonary atresia with ventricular septal defect associated with profound hypoplasia or complete absence of central pulmonary artery, surgical strategy depends on the morphology of native pulmonary artery and major aortopulmonary collateral arteries (MAPCAs). Type of surgery and timing of operation are determined by detailed analysis of angiography and 3-dimensional computed tomography (3D-CT) scan. When native central pulmonary artery exists, palliative procedure like systemicpulmonary shunt or palliative right ventricular outflow tract is recommended in order to promote growth of native pulmonary artery, followed by Rastelli-type definitive repair with/without uniforcalization of MAPCAs. In case of central pulmonary artery absence, staged reconstruction of bilateral pulmonary vascular bed by repeated uniforcalization or 1 stage uniforcalization and Rastelli operation is indicated. However, even after definitive repair, careful attention and scheduled cardiac catheterization with aggressive catheter intervention are required to maintain adequate pulmonary circulation.