中文摘要：

目的探讨成人先天性心脏病（adult congenital heartdisease，ACHD）术后早期临床结果，以提高手术成功率和缩短ICU治疗时间。方法回顾性分析2012年1月至2014年12月阜外医院二部500例ACHD患者临床资料，分别按照是否为复杂畸形及ICU滞留时间是否大于5天进行分组研究。结果患者平均年龄35岁，平均体质量59kg。ICU治疗平均时间2天。术后早期死亡3例。复杂畸形组在年龄、体质量、阻断时间、体外循环时间、呼吸机辅助时间、ICU时间、并发症例数及术中术后用血比例方面与简单畸形组比较差异有统计学意义（P〈0．05）；滞留组在年龄、男性比例、复杂畸形比例、体质量、阻断时间、体外循环时间、呼吸机辅助时间、病死率、并发症例数及术中术后用血例数方面与非滞留组比较差异均有统计学意义（P〈0．05）。结论ACHD病史长，病理生理变化明显，手术及围手术期处理很重要，外科手术可获得满意的效果。专门为ACHD患者服务的专业医疗团队或机构极为重要并亟需建立。

英文摘要：

Objective To summarize 500 cases of surgical experience in restoration of adult congenital heart disease （ACHD） treatment and early postoperative. Methods During January 2012 to December 2014 in Fuwai Hospital, 500 cases of ACHD treated by operation were chosen to collect the clinical data. We divided the groups according to whether the case was a complex malformation and whether the case had an ICU retention time is more than the 5 days. Results The average age was 35, the average weight was 59 kg. The operation average cardiopulmonary bypass（CPB） time was 102min. The average ICU treatment time was 2 days, the average duration of mechanical ventilation was 23 hours, 3 early deaths occurred. The complex malformation group had younger age and less weight than the simple malformation group, the complex malformation group had longer time of cardiopulmonary bypass time, aortic cross clamping time, mechanical ventilation time and ICU treatment time, had higher rate of complication and blood transfusion peri-operative period than the simple malformation group. （ P 〈 0.05 ） The group of ICU retention time less than 5 days had higher rate of the male proportion, had younger age and less weigh, had longer time of cardiopulmonary bypass time, mechanical ventilation time and ICU treatment time, had higher rate of complication and blood transfusion peri-operative period than the control group （ P 〈 0. 01 ）. Conclusion Although ACHD patients have long medical history and complicated pathological and physiological changes, when they get proper surgical operation and periopera- tire treatment, they should obtain satisfied effect. Professional medical team or organization service for the ACHD patient is very important and urgent to build.