Taussig-Bing畸形合并大动脉侧侧位的外科治疗

目的总结外科手术一期治疗Taussig—Bing畸形合并大动脉侧侧位的临床经验。方法2000年5月～2007年9月手术治疗26例（手术年龄3.1±2．2个月）Taussig—Bing畸形合并大动脉侧侧位患者，其中合并主动脉弓病变13例。25例患者采用动脉转位术（arterial switch operation，ASO），1例采用Kawashima手术治疗。合并主动脉弓病变的矫治方法主要是切除缩窄段后，将胸主动脉与主动脉弓下缘行端端吻合，或与升主动脉行端侧吻合。结果住院死亡3例（11.5％，3／26）。2005年6月以后连续手术治疗的15例Taussig—Bing畸形伴大动脉侧侧位，无住院死亡。随访21例，随访时间1～5年；患者生长发育均明显改善，心脏较术前缩小，肺血明显减少。2例患者因肺动脉瓣上狭窄而再次行手术修补。结论Taussig—Bing畸形合并大动脉侧侧位的病理解剖比较复杂，必须根据不同的解剖条件选择不同的手术方法，才能降低手术死亡率。

Surgical Correction for Patients with Taussig-Bing Anomaly with Side by Side Relationship of Great Arteries

Objective To summarize clinical experience of a single stage surgical approach on patients with Taussig-Bing anomaly combined with side by side relationship of great arteries. Methods From May 2000 to Sep. 2007, 26 patients （ age 3.1± 2.2 months） with Taussig-Bing anomaly with side by side great arteries underwent the single stage operation, including arterial switch operation （n = 25）, and Kawashima operation （n = 1）. Aortic arch obstruction was present in 13 patients. The corrections of aortic arch obstruction included descending aorta end to end anastomosis to aortic arch or end to side anastomosis to ascending aorta. Results The hospital mortality rate was 11.5% （3/26）. There was no operative death in continuous 15 patients after Jun. 2005. 21 patients were followed up for 1 to 5 years. The patients＇ growth and development were improved obviously. The sizes of the hearts were smaller than those before operations. The pulmonary blood flow was decreased obviously. Two patients required reoperations because of supravalvular pulmonary stenosis. Conclusion Taussig-Bing anomaly with side by side great arteries has complex anatomical characters. In order to improve the operative outcomes, the optimized operative strategies should be considered in according with different anatomies.