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目的:探讨儿童先天性二尖瓣疾病外科手术的疗效及中期随访结果分析。方法:回顾分析2009年1月至2017年12月在复旦大学附属儿科医院心血管中心接受二尖瓣成形术的患儿92例。其中，二尖瓣狭窄患儿26例(二尖瓣狭窄组)，二尖瓣关闭不全患儿66例(二尖瓣关闭不全组)；男44例，女48例；手术年龄为(42.55±40.57)个月，范围为1个月至14岁，22.8%(21/92)患儿的年龄<1岁。结果:4.3%(4/92)的患儿于手术早期死亡，3.3%(3/92)的患儿在随访期间死亡，总病死率为7.6%。术后所有患儿的心功能分级改善明显，Ⅲ级3例，余均在Ⅱ级及以上，随访时间为(42.72±29.66)个月，随访时间范围为3～104个月。随访期间11例患儿共接受了17次再手术，所有患儿的再手术率为12.0%(11/92)。二尖瓣狭窄组与二尖瓣关闭不全组在生存率及免除再手术率的Log Rank分析中，差异无统计学意义。所有患儿中1岁的患儿具有更高的病死率(n P＝0.045)及再手术率(n P＝0.039)，差异均具有统计学意义。n 结论:二尖瓣成形手术对于治疗先天性二尖瓣疾病的效果显著。但婴幼儿，特别是年龄<1岁的患儿，先天性二尖瓣疾病的修补手术仍面临着巨大挑战。虽然部分患儿需多次手术，瓣膜成形术仍是再手术时首要考虑的手术方式。“,”Objective:To review the surgical approaches and mid-term outcomes of congenital mitral valve diseases in children.Methods:From January 2009 to December 2017, retrospective review was performed for 92 children undergoing mitral valvuloplasty for mitral stenosis (n=26) and mitral regurgitation (n=66). There were 44 boys and 48 girls with an average operative age of (42.55±40.57)(1-168) months. And 21/92(22.8%) were aged under 1 year.Results:Four cases (4.3%) died within an early stage of operation and 3 cases(3.3%) passed away during follow-ups. The total mortality rate was 7.6%. The grade of cardiac function improved significantly in all the children after surgery, including 3 patients with grade Ⅲ, and the rest were grade Ⅱ or above. During a follow-up period of (42.72±29.66)(3-104) months, 11 children (12.0%) underwent 17 reoperations. Log Rank analysis revealed that no significant inter-group difference existed in death or reoperation. Children aged under 1 year had higher mortality and rate of reoperation than those aged over 1 year (n P=0.045 and n P=0.039).n Conclusions:Mitral valvuloplasty is efficacious for congenital mitral valve diseases. However, repairing mitral valves remains a challenge in infants, especially those aged under 1 year. Although some children require multiple reoperations, valvuloplasty is still the most important method of reoperation.