CAJ | 학술논문

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.
목적 토론선천성혈관배적진단화수술치료급영향예후적인소.방법 회고성분석2010년1월지2013년12월수치적42례선천성혈관배환인(점동기수술환인적1.04％)적병례자료.기중남성26례,녀성16례;수술년령24d지6세,평균10.7개월.포괄폐동맥조대26례,쌍주동맥궁10례,우위주동맥궁합병미주좌쇄골하동맥3례,폐동맥조대합병우위주동맥궁병미주좌쇄골하동맥2례,폐동맥조대합병좌위주동맥궁병미주우쇄골하동맥1례.38례환인합병기타적심혈관기형.36례환인행라선CT검사기도중건,23례환인행섬유지기관경검사,제2례기도미견이상외기여환인균존재불동부위불동정도적기관협착혹연화.소유환인균행수술교치선천성혈관배,근거정황동기행심내기형교치수술;1례동기행기관협착절제병Slide성형술,1례재술후행기관내지가식입술.결과 3례환인원내사망(7.1％),사망원인균위술후반복발생기관내육아종형성;기여39례균순리출원.존활환인총체술후호흡궤보조시간14 h(22 h)[M(QR)],심장ICU정류시간5d(8 d),주원시간19 d(9 d).폐동맥조대환인호흡궤보조시간、심장ICU술후정류시간균장우쌍주동맥궁환인[23 h(123 h)비9 h(9 h),7 d(13 d)비4 d(2 d)],단차이무통계학의의(P＞0.05);주원시간명현장우쌍주동맥궁환인[23 d(9 d)비16 d(6 d)],차이유통계학의의(x2=10.157,P=0.006).결론 선천성혈관배조기수술치료시안전、유효적,근기효과량호.합병적기관연화화협착적정도화범위시영향환인예후적관건.
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.