合并双孔二尖瓣的心内膜垫缺损外科手术28例

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

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。

Objective To review the surgical results of 28 cases of endocardial cushion defect combined with double-orifice mitral valve. Methods Of 860 consecutive patients with endocardial cushion defect, double-orifice mitral valve was identiffed 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 postoperative mitral valve dysfunction were reviewed and compared between patients with total endocardial cushion defect （ group Ⅰ, n = 11 ） and partial endocardial 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 stenosis in reoperation or autopsy. There was no later death. Mitral valve function is not satisfactory 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.