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Background Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a relatively rare and extremely heterogeneous form of congenital heart disease. Despite vast improvements in the surgical management of this disease, there is still an ongoing controversy regarding the optimal treatment. The purpose of this chapter is to summarize the surgical algorithm used at Stanford University with a special focus on midline unifocalization. Methods We have an experience with well over 300 patient