合并双孔二尖瓣的心内膜垫缺损外科手术28例

目的 回顾分析28例合并双孔二尖瓣的心内膜垫缺损病儿外科手术效果.方法 1996年10月至2007年11月共860例心内膜垫缺损病儿行矫治手术,28例合并双孔二尖瓣畸形(3.26%),其中完全型心内膜垫缺损11例(组Ⅰ),部分型心内膜垫缺损17例(组Ⅱ).将两组病儿术前、术后有关资料进行对比,包括二尖瓣关闭不全程度以及瓣膜外科处理方法等,并对外科疗效进行分析.结果 术后早期死亡4例,均为组Ⅰ病儿,死于术后严重肺部感染3例,术后低心排1例.2例病儿通过二次手术或尸检证实有二尖瓣严重关闭不全或狭窄.随访过程无中、远期死亡.两组二尖瓣瓣膜外科处理方法无差异,随访3～89个月,平均33个月,组Ⅱ术后远期随访二尖瓣关闭不全程度较组Ⅰ严重,但差异无统计学意义.结论 双孔二尖瓣的存在增加完全型心内膜垫缺损手术风险,影响部分型心内膜垫缺损远期疗效.

Results for repair of 28 cases of endocardial cushion defects combined with double-orifice mitral valve

Objective To review the surgical results of 28 cases of endecardial cushion defect combined with double-ori-rice mitral valve. Methods Of 860 consecutive patients with endocardial cushion defect, double-orifice mitral valve was iden-tiffed in 28 patients (3.26% ) form October 1996 to November 2007. Intracardiac deformities were corrected simultaneously during the operation. Preoperative mitral valve function, surgical procedures and incidence of pestoperative mitral valve dys-function were reviewed and compared between patients with total endecardial cushion defect ( group Ⅰ, n = 11 ) and partial en-docardial cushion defect ( group Ⅱ, n = 17). Results There were 4 operative deaths in group Ⅰ caused by severe pulmonary inflammation in 3 cases and low cardiac output in 1 case. Two cases were identified as severe mitral valve dysfunction or steao-sis in reoperaticu or autopsy. There was no later death. Mitral valve function is not eatisfactory in group Ⅱ as compared with group Ⅰ in 3 to 89 months of follow-up( averaging 33 months). Conclusion Double-orifice mitral valve is a high risk factor for operative death in total endocardial cushion defect and the longer term results of partial endocardial cushion defect combined with double-orifice mitral valve is not satisfactory.