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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1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 summarize the value and experience of transthoracic echocardiography (TTE) preoperative diagnosis and postoperative follow-up in children with mixed total pulmonary venous anomalous drainage (TAPVC). Methods From January 2010 to December 2016, 22 patients with mixed type TAPVC confirmed by surgery in Fuwai Hospital Pediatric Center, Chinese Academy of Medical Sciences, reviewed the data of preoperative and postoperative ultrasound with CT and surgical findings Sexual research. Results The mixed TAPVC in this group accounted for about 5.4% of the total TAPVC. Twenty-two children underwent surgical correction. Among them, there were 19 patients with cardioembolism and cardioembolism and 3 patients with cardia and hypocardioembolism. Among them, 7 cases were complicated with pulmonary venous obstruction (including 1 case of unilateral pulmonary vein dysplasia ), Accounting for a total of about 31.8%. (13/22) 59.1% (13/22) correctly diagnosed before ultrasound, misdiagnosed as cardiac TAPVC in 2 cases, misdiagnosed as intracardiac TAPVC in 5 cases, preoperative missed diagnosis in 1 case as Faruar quadruple Syndrome with pulmonary vein dysplasia in children. The diagnosis rate of obstruction of pulmonary venous return route was (8/13) 61.5%. A total of 12 children (54.5%) underwent enhanced spiral CT before operation. CT was accurate for all pulmonary venous return and the detection rate of pulmonary venous return was 38.5%. All children underwent at least one ultrasound review within 6 months after surgery, with a median follow-up of 4 months. As of the last ultrasound follow-up data showed that 5 cases of postoperative pulmonary hypertension still exist, of which 3 cases of pulmonary veins and left atrial anastomotic or branch stenosis, occurred in 2 months to 4 months. Conclusion Echocardiography can provide important diagnostic clues and follow-up information for mixed TAPVC in children. Being familiar with different types of this disease and understanding the rare variation can improve the diagnostic value of ultrasound, which is beneficial to evaluate the surgical risk and prognosis of children.