本文报告10例经手术证实的心脏型完全性肺静脉异位连接10例,10例均有紫绀。5例临床似房间隔缺损,其右室及肺动脉压力正常或轻度增高,股动脉血氧饱和度为90％左右;另5例临床类似爱森门格氏综合征,右室、肺动脉压力中度或显著增高。股动脉饱和度<88％或<85％。3例死于术后心低排量和呼吸衰竭。最后,讨论了本症的诊断及鉴别诊断。 This article reports 10 cases of surgically confirmed cardiac complete pulmonary vein ectopic connection in 10 cases, 10 cases were cyanotic. 5 cases of clinical atrial septal defect, the right ventricle and pulmonary artery pressure was normal or mildly increased femoral arterial oxygen saturation of about 90%; the other 5 cases of clinically similar Eisenmenger’s syndrome, right ventricular and pulmonary artery pressure Degree or significantly increased. Femoral artery saturation <88% or <85%. 3 patients died of low cardiac output and respiratory failure. Finally, the diagnosis and differential diagnosis of this disease are discussed.