房室管畸形是一种复杂的先天性心脏病,据报道占先天性心脏病的4.2%。对于本病的命名,意见尚未统一,早年多数作者称之为心内膜垫缺损,也有称房室共同通道,原发孔缺损伴二尖瓣裂隙和永存共同房室管等,近年倾向于称为房室管畸形。病理解剖特征房室管畸形的基本病理解剖特征是:(1) Atrioventricular canal deformity is a complex congenital heart disease, reportedly accounting for 4.2% of congenital heart disease. For the naming of the disease, the opinion has not yet unified, the early years of most authors call endocardial cushion defect, also known as common atrioventricular channel, primary defect with mitral valve and permanent co-existence of atrioventricular canal, in recent years tend to call Atrioventricular canal deformity. Pathological anatomy The basic pathological anatomy of atrioventricular canal is: (1)