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目的总结缩窄段切除加主动脉弓补片成形术治疗婴幼儿主动脉缩窄合并主动脉弓发育不良的临床经验。方法 2007年5月至2009年12月,上海交通大学医学院附属上海儿童医学中心对49例婴幼儿主动脉缩窄合并主动脉弓发育不良患者采用主动脉缩窄段切除加主动脉弓补片成形术治疗,其中男30例,女19例;年龄23 d至3岁1个月,<6个月34例,6个月~1岁10例,>1岁5例。31例患者在深低温体外循环下完成手术,采用选择性脑灌注技术;15例在深低温停循环下手术;3例在中低温体外循环下完成手术。31例用自体心包行主动脉弓成行补片,14例采用自体肺动脉壁组织,4例采用异种心包。合并的心内畸形同期纠治。结果围术期死亡1例,死于循环衰竭,手术死亡率2.04%(1/49)。5例术后发生低心排血量综合征,1例合并肾功能衰竭患者,均经相应处理治愈。术后复查超声心动图提示无残余梗阻。随访48例,随访时间4个月至3年。随访期间有1例患者主动脉弓压力阶差>40 mm Hg,计算机断层扫描显示主动脉弓再狭窄,于术后8个月再次手术;2例主动脉弓压力阶差>20 mm Hg,仍在继续随访;其余患者主动脉弓形态良好,与术后当时比较,随访期间主动脉弓降部血流速度无明显变化,计算机断层扫描显示:主动脉弓几何构型正常。术前存在左主支气管受压的患者在主动脉弓成形术后半年,左主支气管受压表现有明显改善或完全消失,无主动脉夹层动脉瘤发生。结论缩窄段切除术加主动脉弓补片成形术是治疗婴幼儿主动脉缩窄合并主动脉弓发育不良的理想手术方法。
Objective To summarize the clinical experience of stenosis and aortic archoplasty for the treatment of infantile aortic constriction with aortic arch dysplasia. Methods From May 2007 to December 2009, Shanghai Children’s Medical Center Affiliated to Shanghai Jiaotong University School of Medicine, 49 cases of aortic constriction with aortic arch dysplasia were treated by aortic stenosis and aortic arch patch angioplasty. There were 30 males and 19 females, aged from 23 days to 3 years and 1 month, 34 cases <6 months, 10 cases of 6 months to 1 year and 5 cases of> 1 year old. Thirty-one patients underwent surgery under deep hypothermic cardiopulmonary bypass with selective cerebral perfusion; 15 underwent deep hypothermic circulatory arrest; and 3 underwent hypothermic cardiopulmonary bypass. 31 cases with autologous pericardial aortic arch into line patch, 14 cases of autologous pulmonary artery wall tissue, 4 cases of heterogenous pericardium. Merged cardiac malformations over the same period correction. Results Perioperative death in 1 case, died of circulatory failure, operative mortality was 2.04% (1/49). 5 cases of low cardiac output syndrome occurred after surgery, 1 patients with renal failure were cured by the corresponding treatment. Postoperative echocardiography showed no residual obstruction. Follow-up 48 cases, followed up for 4 months to 3 years. During follow-up, 1 patient had aortic arch pressure gradient> 40 mm Hg, computed tomography showed aortic arch restenosis and reoperation 8 months after surgery; 2 patients with aortic arch pressure gradient> 20 mm Hg were still under follow-up; the remaining patients The shape of the aortic arch was well. Compared with the time after surgery, there was no significant change in blood flow velocity at the aortic arch during follow-up. Computed tomography showed that aortic arch geometry was normal. Preoperative presence of left main bronchial pressure in patients after aortic arch surgery six months, the performance of the left main bronchus improved or completely disappeared, no aortic dissection aneurysm occurred. Conclusion Narrow resection and aortic arch patch angioplasty are ideal surgical methods for the treatment of infantile aortic constriction with aortic arch dysplasia.