Taussig-Bing畸形合并主动脉弓病变的外科治疗

Surgical treatment for Taussig-Bing malformation with aortic arch disease

目的：总结Taussig-Bing畸形合并主动脉弓病变的手术治疗经验。方法：2012年3月至2017年5月手术治疗5例Taussig-Bing畸形合并主动脉弓病变的患儿，其中主动脉弓中断（Interrupted aortic arch，IAA）3例，主动脉缩窄（Coarctation of aorta，COA）2例。3例患儿采用一期大动脉调转术（Arterial switch operation，ASO）＋IAA矫治术（降主动脉与主动脉弓端侧吻合）；1例患儿分期手术（一期行COA矫治术，二期行ASO手术）；1例患儿行ASO手术＋COA矫治术。结果：全组患儿均顺利出院。气管插管时间平均（158.20±55.64）h；ICU停留时间平均（13.20±3.42）d；住院时间平均（42.00±11.04）d；术后早期并发症包括低心排血量综合征1例、术后出血1例、少尿和腹膜透析1例、肺不张1例。术后随访患儿因残余解剖问题，再次手术者。结论：Taussig-Bing畸形合并主动脉弓病变病理解剖比较复杂，手术方式与合并主动脉弓病变畸形的类型对治疗效果有影响,合并IAA患儿应一期矫治，合并COA的个别患儿可选择二期手术矫治。

Objective To summarize the experiences of surgical treatment for Taussig-Bing malformation with aortic arch disease. Methods 5 patients developing Taussig-Bing malformation with aortic arch diseases were involved. All of these patients received surgical treatment from March 2012 to May 2017.3 cases were inter- rupted aortic arch （IAA）, and the other 2 cases were coarctatiou of aorta （COA）. 3 cases received first-stage arteri- al switch operation plus IAA corrective surgery （anastomosis of descending aorta and aortic arch） ; 1 case received partial stages of operation （first-stage operation was COA corrective surgery; second-stage operation was ASO） ; 1 case received ASO operation and COA corrective surgery. Results All patients successfully discharged from hos- pital. The average tracheal intubation time was （158.20~=55.64 ）hours. The average time length stay in hospital was （42.00~ 11.04）days. The early postoperation complications included 1 case of low cardiac output syndrome, 1 case of postoperative bleeding, 1 case of oligura and peritoneal dialysis and 1 case of atelectasis. None of the pa- tients had anatomical problems remained or needed further operation treatments. Conclusion The Yaussig-Bing malformation with aortic arch disease is a kind of complicated disease for its complicated pathological atonomy. Both of the surgical method chose and the type of the malformation could affect the outcome of treatments. Pa- tients who developed IAA should receive first-stage operation while other patients who developed COA should re- ceive a second-stage operation.