合并主动脉弓离断复杂先天性心脏病同期根治手术的临床疗效  

One -stage repair of the interrupted aortic arch associated with cardiac anomaly

作　　者：闫军[1] 崔彬[1] 刘迎龙[1] 沈向东[1] 李守军[1] 王旭[1] 李巅远[1] 

机构地区：[1]中国医学科学院北京协和医学院心血管病研究所阜外心血管病医院,100037

出　　处：《中华医学杂志》2010年第7期489-491,共3页National Medical Journal of China

摘　　要：目的评价合并主动脉弓离断复杂先天性心脏病同期外科根治手术的临床疗效。方法1996年7月至2008年10月，中国医学科学院北京协和医学院心血管病研究所48例合并主动脉弓离断复杂先天性心脏病患儿接受同期根治手术，其中A型40例，B型8例。男30例、女18例，年龄0．08～7．00（1．97±2．05）岁，体重3～20（9±5）kg；合并中-重度肺动脉高压42例，平均肺动脉压力61～106（82±14）mmHg，肺动脉阻力66—762（315±259）dyn·s·cm^-5，均合并动脉导管未闭，其他合并畸形：室间隔缺损33例、房间隔缺损8例、二尖瓣关闭不全6例、三尖瓣关闭不全5例、主肺动脉窗4例、右室双出口3例、二尖瓣狭窄2例等。手术均采用胸骨正中切口，在全麻深低温低流量、深低温低流量结合停循环、深低温停循环下进行，其中主动脉弓成形术30例，主动脉弓置换术18例，心内畸形同期矫治：室间隔缺损修补术33例、房间隔缺损修补术8例、二尖瓣成形术7例、三尖瓣成形术4例、主肺动脉窗修补术4例、心室内隧道2例、大动脉调转术1例等。结果全组体外循环时间112～375（182±52）min，主动脉阻断时间24～287（99±45）min，气管插管时间8～936（179±133）h，ICU住院时间1～57（14±14）d，出院时复查超声心动图主动脉弓恢复正常解剖结构，无显著压差，心内畸形矫治满意。全组患者死亡3例（3／48，6．25％），死亡主要原因：肺动脉高压危象、呼吸功能衰竭、低心排综合征等。主要并发症：二次开胸止血2例，气管切开2例，术后右侧膈肌麻痹行膈肌折叠术1例，延迟关胸1例，右侧胸腔积液1例，严重肺部感染1例。生存患者均痊愈出院，生活质量提高，随访3个月～12年，无远期死亡或严重术后并发症。结论主动脉弓重建同期矫治心内畸形治疗合并主动脉弓离断复杂先天性心脏病患儿临床疗效满�Objective To evaluate the clinical experiences of one -stage repair of the interrupted aortic arch （IAA） associated with cardiac anomaly in neonates and infants. Methods From July 1996 to October 2008, 48 neonates or infants with IAA associated with cardiac anomaly underwent one-stage repair. There are 30 males and 18 females ranging from 0. 08 to 7. 00 years old（ 1.97 ± 2. 05 ） with body weight 3-20 （9 ± 5 ） kg. 42 patients had the middle-severe pulmonary hypertension （PH） , the pulmonary pressure was 61 - 106（ 82 ± 14） mm Hg and the pulmonary arterial resistance was 66 - 762（315 ± 259） dyn · s · cm^-5 There are 40 patients with type A IAA and 8 patients with type B IAA, all patients had an associated patent ductus arteriosns, other combined malformations included ventricular septal defect（VSD） in 33cases,atrial septal defect （ASD） in 8 cases, mitral valve insufficiency in 6 cases, tricuspid valve insufficiency in 5 cases, aortopulmonary window in 4 cases, double outlet of the right ventricule in 3 cases, mitral valve stenosis in 2 cases, and et aL the operation was performed through median sternotomy under the general anesthesia and cardiopulmonary bypass （CPB, the deep hypothermia with circulatory arrest, the deep hypothermia with low flow rate, or the deep hypothermia with low flow rate and circulatory arrest）, the interrupted aortic arch repair was achieved with the arch anastoutosis in 30 patients and with an interposition graft in 18 patients , the concomitant operations included VSD repair in 33cases, ASD repair in 8cases, mitral valve plasty in 7cases, tricuspid valve plnsty in 4cases, acrtopulmonary window repair in 4cases, intraventricular baffle tube repair in 2 cases, arterial switch operation in 1 case, and et al. Results The time of the CPB and the aortic occlusion were 112 to 375 min（182 ±52） and 24 to 287 min （99 ±45） respectively. The endotracheal intubation time were 8 to 936 hours（ 179 ±133 ） and the ICU stay were 1-57 days （14±14）.

关 键 词：主动脉 胸 心脏病 同期根治手术 

分 类 号：R726.5[医药卫生—儿科]