摘要目的 探讨完全性房室隔缺损(CAVSD)外科治疗的早中期疗效.方法 回顾性分析2009年6月至2016年6月在广东省人民医院心外科接受手术治疗的235例CAVSD患者的临床资料.男性104例,女性131例.手术年龄18 d至58岁,中位数(四分位间距)[M(QR)]为8.9(25.8)个月;其中年龄<3个月26例(11.1％).术前合并无顶冠状静脉窦综合征7例,主动脉缩窄5例,法洛四联症4例,右心室双出口3例,完全性大动脉转位1例,多发肌部室间隔缺损1例,肺动脉吊带1例,十二指肠闭锁或肛门闭锁2例,心肌致密化不全或心肌病2例.手术方式:改良单片法199例,双片法26例,单片法10例;同期或分期实施其他心血管畸形矫治手术.采用广义估计方程统计分析术后二尖瓣和三尖瓣反流情况.结果 18例(7.7％)患者死亡;其中15例为术后早期死亡(6.4％).手术年龄≤3个月的患者病死率最高,为26.9％,不同年龄组间病死率差异有统计学意义(P=0.007),三种手术方式间病死率差异无统计学意义.术后7例患者因重度二尖瓣关闭不全再次手术.217例患者初次术后存活超过1年,术后1年时11例存在重度二尖瓣关闭不全,7例存在重度三尖瓣关闭不全.二尖瓣反流程度在术后1周至6个月呈下降趋势,1年后稍增高;且各手术年龄组中二尖瓣重度反流比例差异有统计学意义(P=0.017),差异主要存在于手术年龄≤3个月组与1～3岁组之间.三尖瓣反流程度术后明显降低,且在随访过程中逐步下降;术后残余重度三尖瓣反流的比例在手术年龄>18岁患者中最高,≤3岁次之,3～18岁最低.结论 年龄≤3个月的CAVSD患者手术病死率明显高于其他年龄段,且术后早中期残余二尖瓣重度反流的比例较高.对小婴儿期实施矫治的CAVSD患者应加强术后随访.改良单片法应用于各年龄段的CAVSD患者都可获得满意的手术效果.

abstractsObjective To discuss the surgical treatment and early-mid follow-up results of complete atrioventricular septal defect ( CAVSD ) . Methods The clinic data of 235 cases of CAVSD underwent surgical treatment at Department of Cardiac Surgery, Guangdong General Hospital from June 2009 to June 2016 was analyzed retrospectively (104 male and 131 female patients). The patients were aged 18 days to 58 years (mid age 8. 9 months) at surgery,and 2 cases of newborns (0. 9％), 24 cases of 1 to 3 months ( 10. 2％) , 56 cases of 4 to 6 months ( 23. 8％) , 66 cases of 7 to 12 months ( 28. 1％) , 36 cases of 1 to 3 years ( 15. 3％) , 37 cases of 4 to 18 years ( 15. 7％) , and 14 cases above 18 years ( 6. 0％) . There were 129 cases of Rastelli type A ( 54. 89％) , 16 cases of type B ( 6. 8％) , 25 cases of type C ( 10. 6％) and 65 cases of transitional type ( 27. 7％) . The patients were combined with 7 cases of unroofed coronary sinus syndrome, 5 cases of coarctation of aorta, 4 cases of tetralogy of Fallot, 3 cases of double outlet right ventricle, 1 case of transposition of the great arteries, 1 case of multiple ventricular septal defect, 1 case of pulmonary artery sling. The CAVSD correction operations were performed in 199 cases with modified single patch technique ( 84. 7％) , 26 cases with two patch technique ( 11. 1％) , 10 cases with conventional single patch technique ( 4. 3％) , and the corrective surgeries of other anomalies were performed in the meantime. Generalized Estimated Equation was used to statistical analysis of postoperative regurgitation level of mitral valve and tricuspid valve. Results Followed-up for 1 to 7 years, 18 cases ( 7. 7％) died overall and 15 cases ( 6. 4％) died within 30 days post-operation. The mortality of little infant ( operation age≤3 months) was much highest(26. 9％).The mortality of different operation age had significant difference ( P=0. 007 ) . There was no difference among the mortality with three techniques. Seven cases ( 3. 0％) suffered from the re-operation for severe mitral regurgitation. A total of 217 patients survived more than 1 year after initial surgery, and there were 11 cases ( 5. 1％) with severe mitral regurgitation and 7 cases ( 3. 2％) with severe tricuspid valve regurgitation at 1 year post-operation. The degree of mitral regurgitation after operation decreased at 1 week to 6 months, and slightly higher after 1 year postoperatively.The ratios of severe mitral regurgitation in different operative age group was statistically significant difference( P=0. 017) , mainly from 3 months group and 1 to 3 years group. The degree of tricuspid regurgitation decreased significantly after operation, and decreased gradually in the follow-up period. The ratio of postoperative residual severe tricuspid regurgitation was the highest in surgical aged >18 years old, less in 3 years group, the lowest in 3 to 18 years group. Conclusions The mortality and the ratio of patients with serious mitral regurgitation were higher in infants less 3 months than elder patients. The infants need more frequently follow-up and medicine treatment after operation. The modified one patch technique is effective for all age groups.