先天性二尖瓣瓣上环的解剖病变及手术效果

目的:总结先天性二尖瓣瓣上环的解剖结构、手术方法和近中期效果。方法:回顾性分析二尖瓣瓣上环病例的临床资料、手术情况、手术前后超声心动图结果及随诊情况。结果:共40例,平均年龄5.0岁,平均体质量16.1kg,其中37例(92%)为中-重度狭窄。Shone复合征(20例)、室间隔缺损(20例)和动脉导管未闭(15例)是最常见的合并心脏畸形。二尖瓣器结构正常和异常的分别为13例(32%,A组)和27例(68%,B组),两组的二尖瓣多普勒超声血流压差和狭窄程度无差别。除B组中1例行机械瓣置换术外,余均行瓣上环切除。B组有17例(63%)需同时处置异常的二尖瓣器。两组共有14例(35%)需同期矫治并发的左心系统狭窄。术后A组二尖瓣血流峰压差(MVPmax)由[(25.8±7.8)mmHg(1mmHg=0.133kPa)降低为(5.8±3.0)mmHg,P=0.0002],B组由[(23.6±5.2)mmHg降为(9.5±4.7)mmHg,P=0.000001],但A组明显低于B组(P=0.005)。两组平均分别随诊2.0和1.6年,无死亡或再次手术病例,A组的MVPmax低于B组[(9.2±4.2)(14.7±6.9)mmHg,P=0.01)],但均高于术后早期(分别为P=0.009和P=0.0002)。结论:大多数先天性二尖瓣瓣上环合并二尖瓣器结构异常,并伴Shone复合征及其他心内畸形,常需同期手术处置。手术近、中期效果良好,但需密切随诊二尖瓣功能,尤其是合并二尖瓣器结构异常者。

The anatomy,surgical management and outcome of supramitral ring

Objective ：To summarize the anatomy, surgical management, early and midterm resuhs of surgical repair of the supramitral ring （SMR）. Methods： The demographic and clinical features, surgical treatmeat, echocardiographic finds before, after the operation and at follow-up in patients with SMR between ]an. 2002 and May 2011 were retrospectively studied. Results： 40 patients aged 5.0 weighted 16. lkg were opera ted, 37 cases （92%） being moderate to severe mitral stenosis （MS）. Shone complex （n =20）, ventricular septal defeat （n = 20） and patent ductus arteriosus （n = 15） were the most associated cardiac defeats. The pa- tients were divided into two groups： cases with relatively normal mitral valve （MV） apparatus except SMR （group A, n =13）, and those with abnormal mitral apparatus （group B, n =27）. The mitral flow Doppler pressure gradients （MVP） and the severity of the MS did not different significantly between the two groups. The SMR were resected in all patients, except one 18 year-old boy with severe MS in group B who was replaced with mechanical valve. Other valvuloplasty procedures in group B including commissurotomy in 12, chordal separa- tion in 2, splitting of the papillary muscle in 3, and elongation of the chordae, resection of the chordae or sutu- ring the cleft of the anterior leaflet in respectively one patient. The maximum MVP （ MVPm= ） decreased from （25.8± 7.8 ） mmHg to （5.8 ± 3.0 ） mmHg （ P = 0.0002 ） in group A, and from （ 23.6 ±5.2 ） mmHg to （ 9.5±4. 7）mmHg （P = 0. 000001 ） in group B, which was much lower in group A （P = 0. 005 ） after the operation. 14 cases （35%） needed correcting the left ventrieular outlet stenosis of Shone complex at the same proce- dure. At a mean follow-up of 2. 0 years in group A, and 1.6 years in group B, there was no death or reopera tion. The MVPmof group A was still lower than group B [ （9. 2 ± 4. 2 mmHg vs 14. 7 ±6. 9 mmHg, P = 0. 01 ） ] , but were both increased than those early after the operation （ P = 0. 009 and P = 0. 0002 ）. Conclusion： Most of the SMR was associated with abnormal MV apparatus. Shone complex and other cardiac defeats often needed to be corrected simultaneously. The early and midterm outcome of surgical correction of the SMR was good, but the MV function should be followed up closely, especially those with abnormal MV apparatus.