中文摘要：

目的：探讨儿童主动脉瓣置换术的病因、瓣膜选择、近远期疗效、死亡和再次手术的危险因素及抗凝相关问题。方法：回顾性分析1990年1月至2014年12月在第二军医大学附属长海医院接受心脏瓣膜置换的62例患儿临床资料,包括男40例,女22例,年龄4~14岁,平均年龄（11.53±2.66）岁,其中先天性心脏瓣膜病41例,风湿性心脏瓣膜病2例,感染性心内膜炎10例,黏液性变心脏瓣膜病9例。62例主动脉瓣置换术均使用机械瓣膜,采用间断褥式缝合方法。部分患者术中采用主动脉根部加宽术（Nicks法4例、Manougnian法11例）。结果：本组住院死亡率为6.5%（4/62）,死亡原因为术后严重低心排综合征3例,术中无法脱离体外循环1例。随访时间1.5~18年,平均（10.2±2.5）年,失访5例,随访率91.4%。随访期间死亡3例,1例为术后9个月死于严重心律失常,1例为术后8年不明原因猝死,1例为术后6年死于感染性心内膜炎。再次瓣膜置换2例。随访期间未发现与抗凝相关的严重并发症,也无因患者-人工瓣膜不匹配再次手术者;患者主动脉瓣膜功能良好,心功能均为Ⅰ~Ⅱ级。结论：儿童主动脉瓣机械瓣置换术近、远期效果良好,大多数患儿可置入较大的瓣膜,术后心脏结构和功能大多能恢复正常。

英文摘要：

Objective：To explore the etiologies,choice for aortic valve replacement,early-and long-term outcomes,risk factors of mortality and reoperation,and anticoagulation treatment of aortic valve replacement in children. Methods：Clinical datas of 62 patients who received aortic valve replacement in Changhai Hospital,between Jan.1990 and Dec.2014,were retrospectively analyzed.There were 40 males and 22 females.The patients were aged from 4 to 14 years,with mean age of（11.53±2.66）years old.The etiologies included congenital heart disease（n=41）,rheumatic valve disease（n=2）,infective endocarditis（n=10）,myxomatous change（n=9）.All 62 patients underwent aortic valve replacement with mechanical prostheses and interrupted mattress sutures to replace the sick valve.The Manougnian procedure was utilized to enlarge aortic valve annular in 11 cases,and the Nicks procedure was utilized in 4 cases. Results：Overall hospital mortality was 6.5%（4/62）.Patients died due to low cardiac output syndrome after operation in 3 cases,and the other died in failure to wean from cardiopulmonary bypass.Follow-up was completed in 53 out of 58 survivors（91.4%）,with a mean follow-up of（10.2±2.5）years（1.5~18 years）.Three patients died and two patients suffered a second valve replacement during follow-up.The 3 deaths owed to endocarditis after 6 years（n=1）,serious arrhythmia after 9 months（n=1）and unknown-cause sudden death after 8 years（n=1）.Twopatients underwent valve replacement.No complications correlated to anticoagulant and prosthesis dysfunction had been found.Nobody underwent reoperation with serious prosthesis-patient mismatch.Cardiac function of patients was at NYHA classⅠorⅡ. Conclusions：Aortic valve replacement with mechanical prostheses can be performed with good early-and long-term outcomes in children.Larger type of mechanical valve could be used in most patients.The operation may lead to normalization of left ventricular size and function in children.