11例先天性主-肺动脉间隔缺损的外科治疗经验体会

11例先天性主-肺动脉间隔缺损的外科治疗经验体会

刘建，马瑞彦，陈林，王学峰，肖颖彬（400037 重庆，第三军医大学新桥医院心血管外科）

[摘要]目的回顾性总结我科2002年8月至2013年5月收治11例14岁以下先天性主-肺动脉间隔缺损（aortopulmonary septal defect，APSD）的诊断及外科治疗经验及疗效。方法本组患者共11例，均为男性，平均年龄6.91 岁（2-14岁），入院后通过心脏超声及多排CT等方法明确诊断，按Mori分型，其中Ⅰ型4例，Ⅱ型4例，Ⅲ型3例，其中5例合并其他心脏畸形，1例继发感染性心内膜炎。结果全组患者术前确诊9例，漏诊2例，均采用胸骨正中切口，9例在中度低温体外循环下完成手术，2例患者采用常温非体外循环下经主肺动脉间隔缺损外游离结扎。术后无早期死亡病例，晚期死亡1例（严重肺部感染及DIC），10例患者术后门诊随访1-10年，症状均明显缓解，复查心脏超声均未见主动脉动脉间隔残余漏。结论术前应通过心脏超声，结合多排CT或者升主动脉造影对APSD明确诊断；合并复杂的心脏大血管畸形与本病的治疗效果密切相关。

[关键词] 主动脉一肺动脉间隔缺损；先天性；外科治疗

Experience of surgical management of aortopulmonary septal defect in 11 cases

LIU Jian，MA Ruiyan，CHEN Lin，W ANG Xuefeng，XIAO Yingbin.Department of cadiac surgery，Xinqiao Hospital，Third Military Medical Univercity，Chongqing，400037 China.

[Abstract] Objective:To review the experience of diagnosis and surgical treatment of 11 patients(admitted from August 2002 to May 2013)with aortopulmonary septal defect(APSD)in our department. Methods:Eleven patients(All males),mean aged 6.91 years(ranged from 2 years to 13 years),were diagnosed by echocardiography and multi slice CT. According to Mori classification system,type I APSD was present on 4 patients,whereas type I and III were found in 4 and3,respectively . Five patients were associated with other cardiac defects,whereas 1 case of infective endocarditis with secondary infection. Results:Correct diagnosis of APSD was made in 9 cases before operation and 2 were misdiagnosed. All patients underwent surgical repair using median sternotomy,cardiopulmonary bypass at moderate hypothermic temperatures was used in 9 cases and three patients underwent ligation without cardiopulmonary bypass. There were no early death and 1 patient was died 28 days after operation because of Severe pulmonary infection and DIC.Range of follow-up was from 1 to 10 years. All 10 surviving operated patients are asymptomatic,with no residual defects by echocardiography. Conclusions:Ultrasound，combined with multi slice CT angiography or ascending aortic angiography are essential to the diagnosis of APSD before operation; Associated complex cardiovascular anomalies are closely related with operative outcomes.

[Key words] aortopulmonary septal defect；congenital；surgical treatment

先天性心脏病发病率占出生活婴的0.4%～1%，其中主动脉-肺动脉间隔缺损是极为少见的一种，占先天性心脏病的0.03%-1%，最早在1830年由Elliotson在一次尸检中发现，表现为升主动脉与肺动脉间存在异常交通，但具有两组独立的半月瓣[1][2]。自2002年至2013年，我科共手术治疗11例此类患者，治疗效果满意，现将结果报道如下：

1病例资料

本组主肺动脉间隔缺损患者共11例，均为男性，平均年龄6.91 岁（2-14岁）。其中9例患者因呼吸道反复感染及发育迟缓就诊，另两例患者发育及活动量正常，仅于参加体检时发现心脏杂音。体检时患儿常表现为左心前区隆起，P2不同程度增强或亢进，4例患儿股动脉枪击音阳性，8例患儿在胸骨左缘3肋间闻收缩期杂音，3例患儿表现为双期连续性杂音；胸部X线片示双肺血增多、肺动脉段不同程度突出，主动脉结小，左室、左房明显扩大或全心扩大。心电图表现与动脉导管未闭相似，表现为左右心室增大。超声心动图提示主-肺动脉间隔有缺损，彩色多普勒可呈现左向右分流或者双向分流，有诊断价值。部分病例需借助心脏64排CT血管造影明确诊断（图1）。分类采用Mori分型：I型4例，II 型4例，III型3例[3]（图2）。合并心内畸形为：动脉导管未闭1例，主动脉弓离断1例，主动脉瓣下隔膜样狭窄1例，右室流出道狭窄1例，房间隔缺损1例，室间隔缺损2例。另有1例患者继发感染性心内膜炎。

2 手术经验及结果

2.1手术方式