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目的:探讨混合型完全性肺静脉异位引流(TAPVC)的形态学特点及手术方式，并分析可能导致术后死亡的危险因素。方法:2011年1月至2019年1月在首都医科大学附属北京儿童医院心脏外科行手术治疗的混合型TAPVC患儿17例。男10例，女7例；年龄(4.4±3.8)个月(1～15个月)；体质量(5.6±1.7) kg(3.5～10.0 kg)；其中1例并室间隔缺损，17例并房间隔缺损，15例并动脉导管未闭。术前并肺静脉狭窄4例，重度肺动脉高压10例。术前5例需呼吸机支持，2例为急诊手术。术前行心脏彩超及心脏CT检查明确诊断。其中Ⅰ型(2+2型)2例，Ⅱ型(3+1型)13例，Ⅲ型(解剖变异型)2例。结果:患儿均行手术治疗，手术原则是将所有肺静脉矫正至左心房。本组患儿体外循环时间(182.3±122.8) min，主动脉阻断时间(84.3±15.9) min，术后呼吸机支持时间(92.9±70.0) h，监护室时间(6.9±4.9) d。本组院内死亡3例(17.6%)，院外死亡1例(5.9%)。结论:混合型TAPVC Ⅲ型死亡率高，术前肺静脉狭窄、重度肺动脉高压及合并心下型是死亡的重要危险因素。手术方式取决于解剖引流方式，建议采用个体化的手术治疗。“,”Objective:To investigate the morphological characteristics and operative methods of mixed total anomalous pulmonary venous connection (TAPVC), and to analyze the risk factors of postoperative death.Methods:From January 2011 to January 2019, 17 cases of mixed TAPVC were operated in Department of Cardiovascular Surgery, Beijing Children′s Hospital, Capital Medical University, with 10 males and 7 females.The average age was (4.4±3.8) months (1-15 months) and the average body mass was (5.6±1.7) kg (3.5-10.0 kg), including 1 case of ventricular septal defect, 17 cases of atrial septal defect and 15 cases of ductus arteriosus.Preoperative pulmonary vein stenosis was discovered in 4 cases and severe pulmonary hypertension was in 10 cases.A total of 5 cases needed ventilator support before operation, and 2 cases needed emergency operation.The diagnosis was confirmed by color Doppler ultrasound and CT before operation.There were 2 cases of type Ⅰ (type 2+ 2), 13 cases of type Ⅱ (type 3+ 1), and 2 cases of type Ⅲ (anatomic variant).Results:All the patients were treated through operation.The principle of operation was to correct all pulmonary veins to the left atrium.The cardiopulmonary bypass time was (182.3±122.8) min, the aortic occlusion time was (84.3±15.9) min, the postoperative ventilator support time was (92.9±70.0) h, and the monitoring room time was (6.9±4.9) d. In this group, 3 cases died in hospital (17.6%) and 1 case died out of hospital (5.9%).Conclusions:The mortality of mixed TAPVC type Ⅲ was high, while preoperative pulmonary vein stenosis, severe pulmonary hypertension and the combination of sub-cardiac type were the important risk factors of death.The operation mode depends on the anatomic drainage mode, so individualized operation is recommended.