婴幼儿主动脉弓中断合并心内畸形胸骨正中切口一期手术治疗  

One-stage surgical repairs for interrupted aortic arch associated with cardiac defects in infants

作　　者：莫绪明[1] 孙剑[1] 彭卫[1] 戚继荣[1] 武开宏[1] 丁晋阳[1] 束亚琴[1] 左维嵩[1] 钱龙宝[1] 

机构地区：[1]南京医科大学附属南京儿童医院心胸外科江苏省小儿先天性心脏病中心,210008

出　　处：《中华胸心血管外科杂志》2012年第12期708-711,共4页Chinese Journal of Thoracic and Cardiovascular Surgery

基　　金：国家自然科学基金（81070137）;国家“十二五”科技支撑计划（201lBAIllB22）;国家卫生行业科研专项（201002006）

摘　　要：目的探讨婴幼儿主动脉弓中断（IAA）合并心内畸形胸骨正中切口一期矫治手术效果以及影响因素。方法2005年8月至2012年1月，采用胸骨正中切口体外循环（CPB）下一期纠治IAA合并心内畸形患婴23例，其中男12例，女11例；年龄18天-3岁，体质量（6．61±3．26）岵。按Rastelli分型，A型20例，B型3例。患儿均合并粗大动脉导管未闭及大型室间隔缺损，其他合并畸形包括主肺动脉窗、右肺动脉起源于主动脉、主动脉瓣两瓣畸形和主动脉瓣下狭窄等。患儿术前均行超声心动图检查，14例行64排CT检查，4例行造影检查。22例术前明确诊断，1例术中明确诊断。行主动脉直接吻合3例，补片扩大主动脉19例，人工管道经肺动脉内连接主动脉弓1例；同期矫治合并心内畸形。结果全组体外循环（129．76±38．98）min，主动脉阻断（74．47±24．30）min，住ICU院平均96h。术后2例死亡，其中1例2月龄患婴死于肺动脉高压危象，1例7月龄患婴术中出血、术后死于严重低心排血量综合征。21例患儿生存，随访2个月-6年，超声检查示患儿术后心功能良好；5例术后6个月上、下肢平均压差〉30mmHg（1mmHg=0．133kPa）。无因再狭窄再次手术患儿。无神经系统并发症发生。结论IAA采用胸骨正中一期外科治疗简化了手术过程，减少了手术死亡，提高了患儿的生活质量，早、中期预后良好，是一种值得推广的治疗方法。Objective To sum up the clinical information and outcomes of one-stage surgical repairs for intemupted aortic arch （IAA） associated with cardiac defects through median stemotomy in infant. Methods From August 2005 to January 2012, 23 IAA patients, aged 18 days to 3 years [ mean age （8.61± 11.81 ） months], body weight 3.3 - 13.0 kg[ mean （6.61 ±3.26） kg] were reviewed underwent one-stage repair. There were 12 male and 11 female. The anatomic subtype in- eluded type A 20 （87.0%） , type B 3（13.0% ） , and no type C in the records. All eases included ventricular septal defect and patent ductus artefious, some also with artirical-pulmonary window, aterical septal defect, bicuspid or unicuspid aortic valve, and subvalvular aortic stenosis. Diagnosis was determined in of the patients and suspected in by echocardiography. Al- so, 64 layers helico-CT was employed to make a definite diagnosis for 18 patients and cardiac catheterization was used for 4 pa- tients. All patients with cardiac anomalies underwent one-stage repair through median sternotomy. The aortic continuity was re- established by anastomosis between the descending aortic segment and aortic arch. Results In all 23 cases, 21 were success- ful. There was 2 （8.69%） postoperative death： one was due to surgical hemorrhage and severe low cardiac output during peri- operative stage and the other was 2-month old due to crisis of pnhnonary hypertension. CPB time was ranged from 53 -215 rain [ （ 129.76 ± 38.98 ） min ], and aortic crossclamp time was 34 - 125 mini （74.47 ± 24.30） min] . The length of stay in ICU postoperatively was 96h averagely. The postoperative complications included severe low cardiac output syndrome in 3 patients, hypoxemia in 13, pneumonia in 7, and supraventricular tachycardia in 12. 21 patients were followed up from 2 months to 6 years and were in good condition without recoactation. Conclusion The outcomes of early and medium term for one-stagerepair of IAA and associated cardiac anomalies through median ste

关 键 词：主动脉弓中断 心脏缺损 先天性 心脏外科手术 婴幼儿 

分 类 号：R726.5[医药卫生—儿科]