婴幼儿混合型完全性肺静脉异位引流的外科治疗

Surgical treatment of mixed total anomalous pulmonary venous connection in infants

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TAPVCⅢ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 typeⅠ(type 2+2),13 cases of typeⅡ(type 3+1),and 2 cases of typeⅢ(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 typeⅢwas 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.