主动脉弓离断21例临床分析  

Clinical analysis of 21 cases of interrupted aortic arch

作　　者：刘芳[1] 黄国英[1] 盛锋[1] 梁雪村[1] 陆颖[1] 吴琳[1] 徐素梅[1] 宁寿葆[1] 

机构地区：[1]复旦大学附属儿科医院心血管中心,上海200032

出　　处：《中国实用儿科杂志》2006年第10期751-752,共2页Chinese Journal of Practical Pediatrics

摘　　要：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 Interruption of the aortic arch （IAA） is defined as absence of luminal continuity between the ascending and the descending aorta. It is a rare but severe malformation, usually with a deteriorative congestive heart failure and high mortality. We analysed the clinical informations of IAA in order to make improvement in the diagnosis and management. Methods The records of 21 IAA patients,aged 12 days to 11 years （ mean age 1.5 months） were reviewed. Results There were ten male and 11 female, accounting for 0. 1% of the patients with congenital heart disease of the same period. The anatomic subtype included type A 18 （85.7% ） ,type B 3 （ 14. 3% ） ,and no type C in the records. All cases were associated with large PDA and VSD,some also with ASD （Ⅱ） ,bicuspid or unicuspid aortic valve,and subvalvular aortic stenosis. Diagnosis was made in 10 of the patients and suspected in 8 by echocardiography. However,echocardiography failed to make diagnosis in 3 （ 14. 3% ）. Surgery repair was pedormed in 9 of the patients. Two older patients were successfully operated in two stages. Seven patients underwent total correction, of which 4 were successful,3 died of ventricular fibrillation,shock due to allergy to protamine sulpate and severe pneumonia during perioperative stage. Conclusion Echocardiography is the primary important method to diagnose IAA, but angiography is necessary for determining diagnosis in some cases. Mortality is relatively higher for totally correction, and the complications during perioperative stage are the important causes of death.

关 键 词：主动脉弓离断 分型 诊断 治疗 

分 类 号：R72[医药卫生—儿科]