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目的 总结经胸超声心动图(TTE)在小儿混合型完全性肺静脉异位引流(TAPVC)术前诊断、术后随访的价值和经验.方法 选择自2010年1月-2016年12月在中国医学科学院北京阜外医院小儿中心经手术证实为混合型TAPVC的22例患者,将术前术后超声资料与CT检查及手术结果对照进行回顾性研究.结果 本组混合型TAPVC约占总体TAPVC的5.4％.22例患儿均成功接受了手术矫治,其中包括:心上型+心内型19例,心上型+心下型3例;其中合并肺静脉回流梗阻7例(含单侧肺静脉发育不良l例),共占约31.8％.超声术前准确探查全部回流途径的病例占(13/22) 59.1％,误判为心上型TAPVC 2例,误判为心内型TAPVC 5例,术前漏诊1例为法鲁氏四联症合并肺静脉发育不良患儿.超声对肺静脉回流途径梗阻部位的诊断率为(8/13) 61.5％.术前共12例患儿(54.5％)接受了增强螺旋CT检查,CT对各支肺静脉回流途径均判断准确,对肺静脉回流梗部位检出率约38.5％.所有患儿术后6个月内均接受至少一次超声复查,中位随访时间为4个月.截止最后一次超声随访资料显示有5例患儿术后仍存在肺动脉高压,其中3例出现肺静脉与左房吻合口或分支狭窄,发生于术后2个月-4个月.结论 超声心动图可为儿童混合型TAPVC提供重要的诊断线索和随访信息,熟悉本病不同类型、了解少见的变异情况有助于提高超声诊断水平,有利于评估患儿手术风险及预后.“,”Objective To explore the important value and diagnostic experience of transthoracic echocardiography (TTE) in preoperative diagnosis and postoperative follow-up of mixed total anomalous pulmonary venous connection (TAPVC) in children.Methods Twenty-two children with mixed TAPVC confirmed by surgery from January 2010 to December 2016 in the Fuwai Hospital of Beijing were reviewed.The Echocardiographic data,CT findings and operation findings were analyzed.Results The relative frequency of mixed type in patient of TAPVC is 5.4％ in our center.All of the 22 cases were successfully treated by operation,including:supracardiac type plus cardiac type in 19 cases,supracardiac type plus infracardiac type in 3 cases.There were 7 cases complicated with pulmonary vein obstruction,31.8％ of 22 patients,including 1 case with unilateral pulmonary vein dysplasia.Echocardiography was performed in each patient,and all the pulmonary veins were diagonised precisely in 13 cases.There were 13 obstructive sites in 22 cases,among them echocardiography recognized 61.5％ sites.Dual Source CT was performed in 12 cases before operation,which detected precisely in every single pulmonary vein,but recognized 38.5％ of the obstructive sites.Follow-up results showed that at least 1 echo was performed in every patient within 6 months after surgery.The median followup time was 4 months(lmonth-6.5years).There were 5 cases who had postoperative pulmonary hypertension during the lastest echo,of which 3 cases were detected pulmonary vein obstruction recurrence.Conclusions Echocardiography can provide important diagnostic and follow-up information for mixed total anomalous pulmonary venous connection in children.Familiar with different anatomic variant of this disease can help echocardiographer to improve the diagnosis during preoperation and follow-up period.