目的：探讨经胸超声心动图（TTE）对主动脉弓离断（IAA）的诊断价值。方法回顾性分析43例各型IAA患儿的超声心动图资料，并与手术或多层螺旋CT血管造影（MSCTA）结果进行对照分析。结果43例患儿中经手术证实为IAA者41例（离断39例，闭锁2例），主动脉缩窄（COA）2例；其中IAA-A型、IAA-B型、IAA-C型所占比例分别为61%（25/41）、32%（13/41）、7%（3/41）。TTE正确诊断35例，误诊3例，漏诊1例，疑诊4例，诊断准确率为81%（35/43）；确诊病例中IAA分型的总诊断准确率为86%（30/35），其中IAA-A型、IAA-B型、IAA-C型诊断准确率分别为96%（23/24）、64%（7/11）、0。MSCTA正确诊断40例IAA，误诊1例，诊断IAA的准确率为98%（40/41）；确诊病例中IAA分型与手术结果一致。41例IAA患儿均合并中重度肺动脉高压和动脉导管未闭，其中39例合并大型室间隔缺损，2例合并主-肺动脉间隔缺损。结论 TTE是诊断主动脉弓离断的首选检查方法，常规行超声心动图检查时，若主动脉及其分支的定位显示不清，联合MSCTA检查可提高对该病诊断的准确率。%Objective To investigate the value of echocardiography in the diagnosis of interrupted aortic arch (IAA). Methods Forty-three children that diagnosed as IAA by transthoracic echocardiography (TTE) were reviewed. The results of CTA (CT Angiography) and operation also were compared. Results Among the 43 children, 41 was admitted as IAA by operation and 2 were diagnosed as coarctation of aorta. According to the type of IAA, 25 cases were diagnosed as type A, 13 cases were diagnosed as type B, 3 cases were diagnosed as type C. Thirty-ifve cases were diagnosed by TTE correctly, 3 cases were misdiagnosed by TTE, 4 cases were suspected as IAA by TTE and ifnally conifrmed by operation. The accuracy rate was 81%(35/43). Among the 35 deifnite diagnosed cases, corrected typing cases were 30, the accuracy rate was 86%(30/35). The accuracy rate of type A, B, C were 96%(23/24), 64%(7/11) and 0. According to the results of CTA, 40 cases were diagnosed correctly, 1 case was misdiagnosed. The accuracy rate was 98%(40/41). Among the deifnite diagnosed cases, corrected typing rate was the same with operation result. Conclusions TTE is the ifrst choice for the detection of IAA. During TTE, in case the indistinct display of aortic arch, CTA should be used to improve the accuracy rate of IAA.
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