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目的 总结 2 0例主动脉弓离断 (IAA)临床资料 ,以期提高对本病的早期正确诊断率。方法 对 1981年 1月至 2 0 0 0年 5月 8481例心血管造影检查中确诊为IAA的 15例患儿以及同期内经尸体解剖确诊为IAA的 5例患儿的临床表现、辅助检查及临床转归进行分析总结。结果 IAA约占先天性心脏病病例中行心血管造影检查者总数的 0 2 %。2 0例患儿中A型占 70 % ,B型占 30 % ,未发现C型病例。除四肢脉搏及血压的改变有一定特异性外 ,其它临床表现、X线胸片及心电图检查等均无特异性。结论 IAA是一种少见的先天性心脏病 ,其生存率与能否早期发现并及时治疗密切相关。如能提高对本病的诊断意识 ,在临床工作中注意提示IAA存在的一些征象 ,辅以适当的实验室检查 ,早期诊断与及时治疗是可以做到的
Objective To summarize the clinical data of 20 cases of aortic arch disconnection (IAA) in order to improve the early correct diagnosis of this disease. Methods From January 1981 to May 2000, 15 children with IAA confirmed by 8481 cardiovascular angiography and 5 children with autopsy confirmed by IAA in autopsy during the same period were included in this study. The clinical manifestations, The outcome of the analysis and summary. Results IAA accounted for about 0.2% of the total number of cardiovascular angiography in congenital heart disease cases. 70 cases of type A in 70 children, 30% of type B, no type C cases were found. In addition to limbs and pulse and blood pressure changes have a certain specificity, other clinical manifestations, X-ray and ECG are non-specific. Conclusion IAA is a rare congenital heart disease whose survival rate is closely related to its early detection and timely treatment. If you can improve the diagnosis of the disease awareness in the clinical work to note some of the signs of IAA, supplemented by appropriate laboratory tests, early diagnosis and timely treatment can be done