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伴有室间隔缺损的肺动脉闭锁(Pulmonary Atresia with VSD)是一种罕见的先天性心脏畸形,有的学者将其称为法乐四联征伴肺动脉闭锁(Tetralogy of Fallot with Pulmonary Atresia)、第Ⅳ型永存动脉干(Truncus Arteriosus Type Ⅳ)[1]、肺动脉缺如(Absence of Pulmonary Artery)或伴有肺动脉缺如的孤立主动脉(Solitary Aorta with Absence of the Pulmonary Art