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目的分析主动脉弓离断(IAA)的发病情况,总结诊断和治疗经验。方法回顾性分析复旦大学附属儿童医院1995—2004年收治的21例IAA患儿的临床资料。结果21例IAA患儿年龄在12d至11岁(中位数1·5个月),男11例,女10例。占同期先天性心脏病患儿的0·1%。其中A型18例(占85·7%),B型3例(占14·3%),无C型病例。所有病例均合并粗大动脉导管未闭及大型室间隔缺损,其它伴发畸形包括继发孔房间隔缺损、主动脉瓣单瓣或两瓣畸形、主动脉瓣下狭窄。超声心动图明确诊断10例,提示IAA或重度缩窄8例,未提示主动脉弓病变3例,漏诊率为14·3%。21例中3例超声心动图诊断比较明确且患儿心功能差,未做心导管检查直接手术治疗,术中发现与超声心动图一致;9例施行手术治疗;2例分期手术均为年长儿,手术效果理想;余7例施行一期根治术(6例为3个月以下婴儿,1例为5岁男孩),其中4例手术顺利,术后恢复好,3例围手术期死亡,死亡原因分别为术后室颤、鱼精蛋白过敏和术后严重肺炎呼吸衰竭。结论超声心动图为初步诊断IAA的重要方法,但有一定漏诊率,确诊宜结合心导管检查及心血管造影;婴儿期治疗采取一期根治术,病死率仍较高,围手术期并发症是导致死亡主要原因。
Objective To analyze the incidence of aortic arch disconnection (IAA) and summarize the diagnosis and treatment experience. Methods The clinical data of 21 cases of IAA admitted to Children’s Hospital of Fudan University from 1995 to 2004 were retrospectively analyzed. Results 21 cases of IAA children aged 12 to 11 years (median 1.5 months), 11 males and 10 females. Accounting for 0.1% of children with congenital heart disease over the same period. There were 18 cases (85.7%) of type A, 3 cases of type B (14.3%), and no type C cases. All cases were complicated with patent ductus arteriosus and large ventricular septal defect, and other associated deformities, including secondary atrial septal defect, aortic valve or two deformities, aortic stenosis. Echocardiography confirmed the diagnosis of 10 cases, suggesting that IAA or severe narrowing in 8 cases, did not prompt the aortic arch lesions in 3 cases, the missed diagnosis rate was 14.3%. 21 cases, 3 cases of echocardiography diagnosis is relatively clear and children with poor cardiac function, cardiac catheterization did not do direct surgery, intraoperative findings and echocardiography; 9 cases underwent surgery; 2 cases were staged surgery were elderly In the remaining 7 cases, radical operation was performed in 7 cases (6 infants less than 3 months old and 1 case 5 years old boy). Of the 4 cases, the operation was successful and the postoperative recovery was good. Three patients died perioperatively, Causes of death were postoperative ventricular fibrillation, protamine allergy and postoperative severe pneumonia respiratory failure. Conclusion Echocardiography is an important method for the preliminary diagnosis of IAA. However, there is a certain misdiagnosis rate. Cardiac catheterization and cardiovascular angiography should be combined in the diagnosis. Infant treatment should be one-stage radical operation with high mortality and perioperative complications The main cause of death.