婴儿期主动脉弓中断的外科治疗

Surgical approaches for interrupted aortic arch in infants

目的回顾总结不同术式治疗婴儿期主动脉弓中断的手术经验和随访结果, 为优化外科治疗方案提供依据。方法收集中国人民解放军总医院第七医学中心2012年10月至2020年9月收治的53例1岁以内的主动脉弓中断患儿临床资料, 其中男38例, 女15例;年龄为(3.0±0.9)个月;体重为(4.0±1.2)kg。53例患儿中, 42例合并室间隔缺损, 1例合并主动脉瓣下纤维环伴重度狭窄、降落伞样二尖瓣、二尖瓣内纤维环, 6例合并主肺动脉窗, 1例合并共同动脉干, 1例合并完全型心内膜垫缺损, 1例合并右室双出口及冠状动脉起源异常, 1例合并主动脉瓣二叶畸形。52例给予一期解剖矫治手术, 1例行分期手术。53例手术患儿中, 行降主动脉与近端主动脉弓单纯端端吻合8例, 行降主动脉与近端主动脉弓扩大端侧吻合28例, 行降主动脉与近端主动脉弓扩大端侧吻合+牛心包补片加宽主动脉弓前壁12例, 行左锁骨下动脉翻转与降主动脉直接吻合2例, 行降主动脉与近端主动脉弓扩大端侧吻合+肺动脉血管补片加宽主动脉前壁3例。记录患儿延迟关胸情况、术后并发症及死亡情况、再狭窄情况等, 采用SPSS 22.0进行统计学分析。结果术后存活48例, 死亡5例。术后延迟关胸26例, 2例术后因低心排血量综合征给予安装体外膜氧合辅助循环治疗;1例术后出现心包填塞, 给予急诊开胸探查止血;1例术后血培养检出金黄色葡萄球菌, 给予亚胺培南联合万古霉素抗感染治疗后痊愈;1例术后出现乳糜胸, 给予禁食辅以肠外静脉营养治疗后痊愈;1例术后出现抽搐, 头颅磁共振成像提示缺氧缺血性脑病, 转神经内科给予营养神经及康复治疗后好转出院。45例随诊病例中, 1例术后1年复查主动脉弓部峰值压差86 mmHg(1 mmHg=0.133 kPa), 遂行经皮介入主动脉球囊扩张治疗, 余随访患儿术后1年及长期随访峰值压差均<30 mmHg。结论患儿一旦确诊主动脉中断应尽早进行手术治疗, 要根据弓中断的部位、范围及合并的心内畸形等个体化选择合理术式。相关并发症如主动脉瓣下隔膜形成及左室流出道梗阻等呈慢性病表现, 长期的随访十分必要, 必要时需再次外科干预。

Objective To summarize the surgical experiences and follow-up results of different surgical approaches for interrupted aortic arch(IAA)and to optimize surgical planning.Methods From October 2012 to September 2020,53 IAA children aged under 1 year were selected as study subjects.The concurrent conditions included ventricular septal defect(VSD,n=41),subaortic membrane and severe stenosis,parachute mitral valve&intramitral annulus(n=1),main aorta-pulmonary window(APW,n=6),common arterial trunk(n=1),complete atrial ventricular septal defect(n=1),double outlet of right ventricle&abnormal origin of coronary artery(n=1)and bicuspid aortic valve(n=1).Primary anatomical correction(n=52)and staged surgery(n=1)were performed.The procedures included end-to-end anastomosis of descending aorta&proximal aortic arch(n=8),extended end-to-side anastomosis of descending aorta&proximal aortic arch(n=28),extended end-to-side anastomosis of descending aorta and proximal aortic arch+bovine pericardial patch widened anterior wall of aortic arch(n=12),left subclavian artery inversion and direct anastomosis of descending aorta(n=2)and extended end-to-side anastomosis of descending aorta and proximal aortic arch and widening of anterior aortic wall with pulmonary vascular patch(n=3).The parameters of delayed sternal closure time,postoperative mortality and restenoticc rate were statistically analyzed with SPSS 22.0.Results The outcomes were survival(n=48),death(n=5)and delayed thoracic closure(n=26).Two postoperative children underwent extracorporeal membrane oxygenation(ECMO)assisted circulation due to low cardiac output syndrome.One case of postoperative cardiac tamponade underwent emergency thoracotomy for hemostasis;Staphylococcus aureus was detected in blood culture of 1 child and cure obtained after antibiotic dosing of imipenem and vancomycin.In another case,chylothorax disappeared after a combination of fasting and with parenteral nutrition.One child developed postoperative convulsions and cranial MRI hinted at hypoxic-ischemic encephalopathy.After intensive nutrition and rehabilitation,he was discharged after improvements.One child underwent percutaneous balloon dilation of aortic arch with a pressure difference of 86 mmHg(1 mmHg=0.133 kPa)at 1 year postoperatively.And others were followed up with a pressure difference of<30 mmHg for 1 year.Conclusions Upon a definite diagnosis of IAA,early surgical intervention is mandatory.Proper surgical approaches should be based upon the location,extent and associated intracardiac abnormalities.Surgical intervention is indicated for such chronic conditions as subaortic membrane formation and left ventricular outflow obstruction.