新生儿主动脉弓中断一期矫治的疗效及随访结果

Single-stage repair of interrupted aortic arches and associated cardiac anomalies in neonatal:the outcomes and follow-up

目的总结一期矫治新生儿主动脉弓中断(IAA)合并畸形的疗效及随访结果。方法2012年10月至2019年6月,我院心脏中心一期外科手术纠治新生儿IAA 38例,男28、女10例;手术年龄(12±7)天;体质量(3.3±0.5)kg。按Celoric和Patton分型:A型29例(76%)、B型7例(18%)、C型2例(6%)。其中合并室间隔缺损30例,右心室双出口(Taussig-Bing畸形)4例(10.5%),主动脉瓣二叶瓣畸形4例(10.5%),左心室流出道狭窄(瓣下隔膜)1例(2.6%),永存动脉干1例(2.6%),主肺动脉窗及新生儿坏死性小肠结肠炎1例(2.6%)。38例主动脉弓离断患儿,均行一期解剖矫治手术。手术均经正中切口,于体外循环深低温停循环或低流量脑灌注下进行,彻底充分切除导管组织,采用降主动脉近心端与主动脉弓远心端扩大端侧吻合。同期进行室间隔缺损、主肺动脉窗修复,对伴右心室双出口行大动脉调转、永存动脉干行REV等手术纠治。结果术后早期死亡2例(5.2%),1例为IAA合并右心室双出口患儿术后24 h内死于低心排血量综合征;1例术后20天死于肺脓肿、感染性休克。延迟关胸12例(31%),腹膜透析8例(20%)。中位体外循环时间138 min(90~305 min),中位主动脉阻断时间68 min(47~163 min),中位停循环时间24.5 min(13.5~61.0 min),中位手术时间248 min(180~390 min)。术后患儿呼吸机辅助通气4~18天,中位值9天;住院13~52天,中位值25天。36例顺利出院,出院时上下肢血压差均小于20 mmHg(1 mmHg=0.133 kPa)。术后随访32例,随访率87.5%,中位随访时间34个月(9~85个月),2例(6.0%)肺动脉或分支肺动脉轻度狭窄(压差20~25 mmHg),3例(9.3%)主动脉弓吻合口轻度狭窄(压差21~44 mmHg),1例(3.1%)左心室流出道狭窄(瓣下隔膜狭窄),进行性加重,术后10月返院行左心室流出道疏通术。1例(3.1%)左支气管轻度狭窄,为IAA合并主肺动脉窗,无临床症状,无需外科处理。全组心肺功能良好。结论新生儿主动脉弓中断合并畸形一期矫治成功率高,死亡比例低,手术安全,近期效果良好,远期效果待随访。

Objective This study defined mid-term results of a policy of single-stage repair of interrupted aortic arch associated cardiac anomalies.Methods Between October 2012 and June 2019,38 patients with interrupted aortic arch were evaluated for short-and mid-term results after surgical treatment,the average age of patients was(12±7)days and the mean body weight was(3.3±0.5)kg.29 patients belonged to IAA type A,and 7 patients to type B,and 2 patients belonged to IAA type C,30 patients were complicated with ventricular septal defect,atrial septal defect,and patent ductus arteriosus,two complicated with bicuspid aortic valve,four complicated with Taussig-Bing malformation and two with double outlet right ventricle,one with truncus arteriosus(A4),one with aortopulmonary window,one with left ventricle outflow tract obstruction.Single-stage repair was performed in all patients with cardiac anomalies.Results Early mortality was 5.2%(2 cases),one was die for low cardiac output syndrome,the other die of septic shock.Median CPB time was 138 min(90-305 min),and median aortic cross clamping time was 68 min(47-163 min).Hospital day was 25d(13-52 days).32 patients were followed-up,median time 34 months(9-85months).2 patients(6.0%)were mild pulmonary stenosis pressure(20-25 mmHg),3 patients(9.3%)suffer aortic anastomosis mild stnosis(21-44)mmHg.1 patient(3.1%)LVOTO was repaired ten months after the repair of interrupted aortic arch.The left broncus of of one patient was mild stenosis but without dyspnea.Conclusion Single-stage end-to-side anastomosis repair of interrupted aortic arches in neonatal and repair of associated cardiac anomalies is safe and have low effective with low motality.