大动脉调转术同期行主动脉弓重建在大动脉转位 合并主动脉弓病变患者中的疗效——单中心经验

Outcomes of the single-stage arterial switch operation and aortic reconstruction in patients with transpo⁃sition of great arteries and aortic arch obstruction——single center experience

目的评估单中心行大动脉调转术及主动脉弓重建一期矫治大动脉转位(transposition of the great arteries,TGA)或右心室双出口肺动脉瓣下室间隔缺损(Taussig-Bing)合并主动脉弓梗阻病变的手术疗效及预后。方法回顾性分析2011年1月到2021年12月,在广东省妇幼保健院进行动脉调转术和主动脉弓重建一期矫治的18例TGA或右心室双出口肺动脉瓣下室间隔缺损合并主动脉梗阻病变患者的术后近中期随访结果。结果18例患者均完成治疗,其中男14例,女4例。12例为右心室双出口肺动脉瓣下室间隔缺损,6例为TGA。主动脉弓部病变:主动脉缩窄14例,主动脉弓离断4例。患儿手术时的年龄为13(2~88)d,体质量为(3.5±0.8)kg。手术早期死亡2例,占11.1%。随访49(14~125)个月,随访期间无死亡患者。所有患者均行大动脉调转术及主动脉弓梗阻一期矫治:体外循环时间为(254±52)min,主动脉阻断时间为(124±23)min,术后住院时间为26(2~36)d。3例患者行再次手术干预(18.7%,3/16):1例患者为主动脉及右肺动脉狭窄,行经皮球囊成形术,效果欠佳,行外科手术治疗;1例患者行经皮主肺动脉球囊成形术,效果满意;1例主动脉吻合口狭窄患者,行经皮主动脉球囊成形术,效果满意。实际1年和5年免于再手术比例分别为100%和77%(95%CI:71%~91%),无再手术死亡患者。结论大动脉调转术及主动脉弓重建一期矫治TGA或右心室双出口肺动脉瓣下室间隔缺损合并主动脉弓梗阻病变患者的手术效果满意,有一定的再干预率,远期疗效需持续密切随访。

Objectives To evaluate the surgical effect and prognosis of patients with transposition of great arteries and⁃double outlet right ventricular with sub-pulmonary valve ventricular septal defect(Taussig-Bing)undergoing arterial switch operation and aortic arch obstruction repair.Methods Results of near and medium-term follow-up after operation in 18 patients with TGA and Taussig-Bing undergoing arterial switch operation and aortic arch obstruction from January 2011 to December 2021 in Guangdong Maternal and Child Health Hospital were retrospectively analyzed.Results The 18 patients were completed treatment,including 14 males and 4 females.Twelve patients had Taussig-Bing and 6 patients had TGA.Aortic arch obstruction included aortic coarctation in 14 cases and interrupted of aortic arch in 4 cases.Median age of the patients at surgery was 13(2-88)days and mean body weight was(3.5±0.8)kg.There were 2 early deaths(11.1%,2/18).Follow-up was available for 16 survivors.Median follow-up duration was 49(14-125)months.There were no late deaths.All the 18 patients underwent arterial switch in single-stage correction of aortic arch obstruction:cardiopulmonary bypass duration was(254±52)min,aortic crossclamp duration was(124±23)min,hospital stay duration was 26(2-36)days.Three patients underwent intervention(18.7%,3/16):in 1 patient with aortic and right pulmonary artery stenosis,percutaneous balloon angioplasty was performed with unsatisfied results,and surgical treatment was performed;another patient underwent percutaneous balloon angioplasty had satisfied outcome;the third patient with aortic stenosis underwent percutaneous aortic balloon pasty had satisfied outcomes.Freedom from reintervention was 100%and 77%at 1,5years,respectively.No reoperation death.Conclusions Arterial switch and aortic arch reconstruction in single-stage procedure for the treatment of patients with TGA or Taussig-Bing and aortic arch obstruction have satisfactory outcomes with a certain reintervention rate.The long-term efficacy of the patients should be obtained with continuous and close follow-up.