儿童先天性主动脉缩窄的解剖特点及外科治疗策略

The anatomical variations and surgical treatment of congenital coarctation of aorta in children

目的：探讨儿童先天性主动脉缩窄（CoA）的解剖变异特点及相应外科治疗策略。方法回顾性分析2012年5月—2014年6月南京医科大学附属儿童医院心胸外科手术治疗的30例先天性 CoA 患儿的临床资料,其中男22例,女8例；年龄12 d ～6岁。依据国际小儿心脏外科命名和数据库的分类标准：单纯型 CoA 12例,合并室间隔缺损型8例,合并复杂先天性心脏病型5例,合并主动脉弓发育不良型5例。30例患者中,体外循环下手术矫治20例,同期矫治心内畸形,其中8例在选择性脑灌注下行手术矫治；非体外循环手术10例,2例采用正中切口,其中1例采取镶嵌手术封堵肌部室间隔缺损,其余8例采用左后外侧切口。结果30例患者均顺利完成手术,手术时间95～675 min,平均165.68 min。术后死亡2例；1例术后出现气道压力增高,左上肺不张,不能脱离呼吸机,再次行手术悬吊松解,顺利脱离呼吸机并出院；余27例患儿均顺利康复并出院。28例患儿获随访,随访时间6个月～2年,其中1例于术后2年出现再次狭窄,上下肢血压压差在50 mmHg （1 mmHg =0.133 kPa）左右,目前仍在随访中；余患儿生长发育正常,术后上下肢血压压差＆lt;10 mmHg。结论对先天性 CoA 患儿,根据 CoA 不同的解剖分型选择相应的外科治疗及体外循环方案,可取得良好的治疗效果。

Objective To investigate the anatomical variations and surgical treatment of congenital coarctation of aorta in children. Methods From May 2012 to Jun 2014, 30 patients （male 22, female 8） between the ages of 12 days to 6 years with congenital coarctation of aorta who received surgical treatment in department of Cardiothoracic Surgery of Nanjing Children＇ s Hospital were reviewed. According to the international classification criteria for Pediatric Cardiac Surgery Nomenclature, anatomical variations included simple type coarctation （12 patients）, coarctation with ventricular septal defect （ VSD, 8 patients）, coarctation with complex congenital heart disease （5 patients）, coarctation with hypoplasia of aortic arch （5 patients）. Among the total 30 patients, 20 patients were performed under cardiopulmonary bypass and 8 with the assistance of selective cerebral perfusion, 10 patients were performed without cardiopulmonary bypass, included 2 patients which were carried out through median sternotomy （1 with hybrid occlusion of muscular VSD） and the other 8 patients which were done through left thoracic incision. Results All the patients received the surgical correction of coarctation of aorta and associated cardiac anomalies successfully, surgical duration from 95 to 675 min, mean 165. 68 min, 2 patients were dead in this group. One patient was reoperated （surgical sling） because of his high peak airway pressure and being difficult to wean from mechanical ventilation, and he was successfully weaned from respirator and discharged from hospital after reoperation. All the other patients recovered uneventfully and discharged from hospital after recovery. Follow up （0. 5 - 2 years） information of 28 survived patients showed 1 patient with restenosis was found, whose pressure difference between upper/ lower limbs was 50 mmHg（1 mmHg = 0. 133 kPa） and he was still under close follow up, the other patients had normal growth and development, whose pressure difference between upper/ lower limbs was less than 10 mmHg. Conclusions Excellent clinical results can be achieved in the children with congenital coarctation of aorta, who underwent different surgical treatments and suitable cardiopulmonary techniques according to the anatomical variations.