11例先天性主-肺动脉间隔缺损的外科治疗临床分析

Surgical management of aortopulmonary septal defect:clinical analysis on 11 cases

目的 回顾性总结本科2002年8月至2013年5月收治的11例14岁以下先天性主-肺动脉间隔缺损患儿的诊断及外科治疗经验及疗效。 方法 本组患儿共11例，均为男性，平均年龄6.91（2～14）岁，入院后通过心脏超声及CTA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 全组患儿术前确诊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例患儿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（严重肺部感染及弥散性血管内凝血），10例患儿术后门诊随访1～10年，症状均明显缓解，复查心脏超声均未见主-肺动脉间隔残余漏。 结论 术前通过心脏超声，结合CTA或者升主动脉造影对先天性主-肺动脉间隔缺损可明确诊断；合并复杂的心脏大血管畸形与本病的治疗效果密切相关。

Objective To retrospectively analyze our experience in the diagnosis and surgical treatment of aortopulmonary septal defect （APSD） in our department on 11 patients from August 2002 to May 2013. Methods Eleven patients, all males at a mean age of 6.91 years （ranging from 2 to 13 years）, were diagnosed as APSD by echocardiography and multi-slice CT scanning. According to Mori classification system, type Ⅰ APSD was presented on 4 patients, whereas type Ⅰ and Ⅲ were found in 4 and 3, respectively. Five patients were associated with other cardiac defects, whereas 1 case had secondary infective endocarditis. Results Correct diagnosis of APSD was made in 9 cases before operation and 2 were misdiagnosed. All patients underwent surgical repair using median sternotomy, and 9 of them received cardiopulmonary bypass at moderate hypothermic temperatures, and 3 underwent got without cardiopulmonary bypass. There was no case died early, and 1 died in 28 d after operation because of severe pulmonary infection and DIC. The left 10 patients were followed up from 1 to 10 years. All of them survived with obvious remission of symptoms. Echocardiography showed there was no residual defects. Conclusion Echocardiography should be carried out and combined with multi-slice CT angiography or ascending aortic angiography to diagnosis of APSD before operation. Associated complex cardiovascular anomalies are closely related with operative outcomes.