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

Patch Aortoplasty for Infant Coarctation of the Aorta with Hypoplastic Aortic Arch

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 experiences of resection with patch aortoplasty for infant coarctation of the aorta combined with aortic arch hypoplasia.Methods Between May 2007 and December 2009,49 patients including 30 males and 19 females with coarctation with hypoplastic aortic arch underwent coarctation resection and patch aortoplasty in Shanghai Children＇s Medical Center,School of Medicine,Shanghai Jiaotong University.The age of the patients ranged from 23 days to 3 years and 1 month with thirty-four patients under 6 months,ten between 6 months and 1 year old,and five more than 1 year old.The surgery under deep hypothermia cardiopulmonary bypass with selective cerebral perfusion were performed in 31 cases and circulation arrest in 15 cases;under moderate hypothermia cardiopulmonary bypass in 3 cases.Pericardia patch was used in 31 cases,pulmonary autograft patch in 14 cases and xenograft pericardia patch in 4 cases.The associated intracardiac anomalies were repaired in the same stage.Results One case died from circulation failure during the perioperative period.The operative mortality was 2.04%（1/49）.Low cardiac output syndrome and renal failure respectively occurred in 5 cases and 1 case who were cured afterwards by correspondent treatments.No residual obstruction was detected by echocardiography after the operation.Follow-up was carried out in forty-eight cases for a minimum of 4 months and a maximum of 3 years.Echocardiographic examination showed that the gradient through the aortic arch was more than 40 mm Hg and computed tomography showed recoarctation in 1 case who underwent reoperation eight months after the operation;the gradient was more than 20 mm Hg in 2 cases who were under continuous observation;all the rest cases had a fine aortic arch morphology and for these patients,the blood velocity at descending aortic arch was not obviously changed during the follow-up period compared with that right after operation,the computed tomography showed a normal aortic arch geometry.Left bronchus compression was relieved obviously or totally disappeared in patients who suffered from left bronchus stenosis before the operation without any aortic aneurysm detected.Conclusion Coarctation resection with patch aortoplasty is considered as an optimal surgical method for management of infant coarctation with hypoplastic aortic arch.