先天性血管环的外科治疗

Surgical treatment of congenital vascular ring

目的 讨论先天性血管环的诊断和手术治疗及影响预后的因素.方法 回顾性分析2010年1月至2013年12月收治的42例先天性血管环患儿（占同期手术患儿的1.04％）的病例资料.其中男性26例,女性16例;手术年龄24d至6岁,平均10.7个月.包括肺动脉吊带26例,双主动脉弓10例,右位主动脉弓合并迷走左锁骨下动脉3例,肺动脉吊带合并右位主动脉弓并迷走左锁骨下动脉2例,肺动脉吊带合并左位主动脉弓并迷走右锁骨下动脉1例.38例患儿合并其他的心血管畸形.36例患儿行螺旋CT检查气道重建,23例患儿行纤维支气管镜检查,除2例气道未见异常外其余患儿均存在不同部位不同程度的气管狭窄或软化.所有患儿均行手术矫治先天性血管环,根据情况同期行心内畸形矫治手术;1例同期行气管狭窄切除并Slide成形术,1例在术后行气管内支架植入术.结果 3例患儿院内死亡（7.1％）,死亡原因均为术后反复发生气管内肉芽肿形成;其余39例均顺利出院.存活患儿总体术后呼吸机辅助时间14 h（22 h）[M（QR）],心脏ICU停留时间5d（8 d）,住院时间19 d（9 d）.肺动脉吊带患儿呼吸机辅助时间、心脏ICU术后停留时间均长于双主动脉弓患儿[23 h（123 h）比9 h（9 h）,7 d（13 d）比4 d（2 d）],但差异无统计学意义（P＞0.05）;住院时间明显长于双主动脉弓患儿[23 d（9 d）比16 d（6 d）],差异有统计学意义（x2=10.157,P=0.006）.结论 先天性血管环早期手术治疗是安全、有效的,近期效果良好.合并的气管软化和狭窄的程度和范围是影响患儿预后的关键.

Objective To discuss the diagnosis and surgical treatment of congenital vascular ring and prognostic factors.Methods The clinic data of 42 cases of congenital vascular from January 2010 to December 2013 was analyzed retrospectively （accounting for 1.04% congenital heart operations over the same period）.There were 26 male and 16 female patients,aged at surgery 24 days to 6 years （average 10.7 months）.The diagnosis including pulmonary artery sling in 26 cases,double aortic arch in 10 cases,right aortic arch with aberrant left subclavian artery in 3 cases,pulmonary artery sling and right aortic arch with vagus left subclavian artery in 2 cases,pulmonary artery sling and left aortic arch with vagus right subclavian artery in 1 case.In addition to 4 cases the remaining 38 patients were still combined with other cardiovascular malformations.Thirty-six cases of children underwent spiral CT airway remodeling,23 children underwent fiber-bronchoscopy.In addition to 2 cases of airway abnormalities not seen,the rest of the children were present in varying degrees in different parts of tracheal stenosis or tracheomalacia.All patients underwent surgical correction of congenital vascular ring,concomitant heart deformity correction surgery according to the situation （7 cases of atrial septal defect repair,3 of ventricular septal defect repair,1 of mitral valvuloplasty,1 of bi-Glenn,1 of coarctation of the aorta correction with ventricular septal defect repair,1 of trilogy of Fallot correction）.One case suffered resection of tracheal stenosis and Slide tracheoplasty simultaneously,another case was implanted tracheal stenting postoperatively after pulmonary artery sling correction.Results Three patients died in hospital （7.1%）,the cause of death were recurrent granuloma formation postoperatively.Remaining 39 patients were successfully discharged.The median time of overall survival of children with mechanical ventilation was 14 h （22 h） （M（QR））,median ICU residence time was 5 d （8 d）,and the median in-hospital time was 19 d （9 d）.Tracheal intubation time and postoperative ICU duration time in children with pulmonary artery sling children were much more than in children with double aortic arch （23 h （123 h） vs.9 h（9 h）,7 d （13 d） vs.4 d （2 d））,but the difference were not statistically significant.There was significant difference in the duration of hospitalization between the patient with the pulmonary artery sling and double aortic arch （23 d （9 d） vs.16 d（6 d）） （x2 =10.157,P =0.006）.Conclusions The recent results of surgical treatment of congenital vascular ring is safe and effective.The extent and scope of tracheal stenosis and tracheomalacia is a critical influence prognosis.