107例大动脉转换术的冠状动脉解剖类型和治疗结果

目的总结分析近年来107例大动脉转换术的冠状动脉解剖分类和手术结果，以进一步提高大动脉转换术的手术成功率。方法2000年1月至2004年9月，采用大动脉转换术纠治完全性大血管错位室隔完整型（TGA／IVS）44例，完全性大血管错位伴室间隔缺损（TGA/VSD）38例，右室双出口伴肺动脉瓣下室间隔缺损、肺动脉高压（Taussig-Bing）25例，其中冠状动脉畸形28例，占全组28％。结果大动脉转换术107例中死亡17例，总病死率15．88％。其中TGA/IVS组死亡4例，病死率9．02％；TGA／VSD组死亡8例，病死率21．05％；Taussig-Bing组死亡5例，病死率20．00％。90例术后随访6个月～4年，VSD残余漏3例，2例分别在术后1个月和2个月自愈，1例同时伴右室流出道梗阻，术后3个月再次手术治愈。肺动脉瓣上狭窄2例尚在随访中。其余病儿活动良好，无任何心肌缺血表现。结论冠状动脉畸形的变异很多，分型比较困难，Leiden方法简单，易掌握。Taussig-Bing的冠状动脉畸形发生率较高，TGA／VSD的冠状动脉畸形达40％左右，进行Switch手术时应注意。     

动脉转位术的临床应用

目的 总结动脉转位术(arterial switch operation，ASO)治疗完全型大动脉转位(transposition of the great arteries，TGA)和右心室双出口伴肺动脉瓣下室间隔缺损(VSD)的临床经验。方法 采用ASO治疗小儿先天性心脏病32例，其中TGA22例，伴室间隔完整型(intact ventricular septum，IVS)9例，伴VSDl3例；右心室双出口伴肺动脉瓣下VSD(Taussig—Bing)10例。结果TGA／IVS9例中死亡1例，TGA／VSD13例中死亡4例，Taussig-Bing10例死亡3例，总手术死亡率25％(8／32)。术后随访3个月～2年，所有患者紫绀消失，活动能力明显增强。1例Taussig—Bing术前二尖瓣轻-中度反流，术后仍为中度反流；2例TGA主动脉和肺动脉瓣上狭窄，压差40mmHg(1kPa=7．5mmHg)，1例肺动脉瓣下狭窄和残余VSD，3个月后再次手术治愈。结论 ASO已广泛应用于TGA的纠治，手术效果满意；应用于右心室双出口肺动脉瓣下VSD的早期纠治，不但可防止发生肺血管阻塞性病变，而且避免了心内修补左心室流出道梗阻的远期并发症。     

大动脉转换术治疗新生儿完全性大动脉错位

目的 回顾总结完全性大动脉错位（D－TGA）的手术治疗。方法 1999年11月至2001年8月，采用大动脉转换术（Switch）手术方法纠治新生儿D－TGA 16例，其中室间隔完整型大动脉错位（D－GA／IVS）6例，大动脉错位伴室间隔缺损（D－TGA／VSD）10例。所有病例都在深低温低流量和深低温停循环下行Switch纠治术。结果 Switch手术治疗TGA共16例，其中TGA／IVS 6例，无死亡；TGA／VSD 10例，死亡2例，均为伴有冠状动脉畸形者。14例随访2－20个月，VSD残余漏1例，肺动脉轻度残余梗阻2例，14例心功能均良好。结论 Switch手术是D－TGA纠治的首选方法，但必须早期手术。     

完全性大血管错位和右心室双出口肺动脉瓣下室间隔缺损伴主动脉弓病变的一期矫治手术

目的评估一期纠治完全性大血管错位(TGA)和右心室双出口肺动脉瓣下室间隔缺损(Taussig-B ing)伴主动脉弓病变的手术疗效。方法2001年1月—2004年6月对8例伴主动脉弓病变的TGA(3例)和Taussig-B ing(5例)行一期手术治疗。3例TGA中,室间隔完整型1例,伴室间隔缺损2例;主动脉弓病变为7例主动脉缩窄、1例主动脉弓中断。手术年龄1例为8个月,7例为5 d~3个月,平均40 d,体重3.5~6.3 kg,平均(4.3±0.5)kg。均采用胸骨正中切口。手术先在深低温、停循环下矫治主动脉弓病变,然后在深低温、低流量下行大动脉转换术(Sw itch术)。体外循环转流时间107~159 m in,平均(126±23)m in,主动脉阻断时间63~118 m in,平均(92±16)m in,停循环14~45 m in,平均(30±12)m in。结果手术死亡1例,为8个月Taussig-B ing伴主动脉弓发育不良、冠状动脉畸形患儿,术后因低心排血综合征、Ⅲ度房室传导阻滞、肺高压危象死亡;1例3月龄患儿术后5 d喂奶时窒息死亡。6例随访5个月~2年,生长发育良好,1例Taussig-B ing主动脉弓中断出现吻合口狭窄,压差60 mm Hg;2例出现主动脉瓣轻微返流,1例肺动脉瓣轻度返流。结论一期纠治TGA和Taussig-B ing伴主动脉弓病变能取得较好手术效果,手术死亡原因为肺动脉高压和冠状动脉畸形。     

完全性大动脉转位的麻醉处理

完全性大动脉转位(TGA)是比较常见的紫绀型先天性心脏病(简称先心病)之一,约占先心病的7%~9%,自然预后极差。大动脉转换术又叫大动脉调转术(switch术),已广泛应用于完全性大血管转位的纠治,取得较好的手术效果[1,2]。现将我院自1998年5月至2004年11月共8例患儿完全性大动脉转位     

大动脉转换术113例的手术结果分析

目的回顾性分析大动脉转换术的手术疗效。方法2001年1月至2005年12月,采用大动脉转换术纠治完全性大动脉错位113例,其中室间隔完整型大动脉错位（TGA／IVS）60例,伴室间隔缺损大动脉错位（TGA／VSD）53例。患儿体重最轻2,3kg,年龄最小出生后6h。在深低温停循环和低流量下行大动脉转换术。结果手术总死亡率9．7％,其中TGA／IVS死亡5例,手术死亡率8．3％,TGA／VSD死亡6例,手术死亡率11.3％。随着手术方法的不断改进和围手术期以及体外循环转流技术的提高,其手术死亡率不断下降,从早期的手术死亡率16.6％降至目前的5．6％。结论冠状动脉畸形是导致大动脉转换术死亡的关键,TGA／VSD的冠状动脉畸形发生率较TGA／IVS要高。主动脉和肺动脉的位置并不影响手术成功率。左心室与右心室压力比例〈0．6时,不能行大动脉转换术,否则术后将出现严重左心室低心排。     

大动脉转换术治疗右室双出口合并肺动脉瓣下室间隔缺损

目的：采用大动脉转换术治疗右室双出口合并肺动脉瓣下室间隔缺损（Taussin-Bing综合征）。方法：5例2－7月龄，体重4．5－6．5kg的Taussig-Bing综合征病儿经大动脉转换术纠治。其中2例因肺炎，心衰急诊手术。所有病例在深低温停循环或低流量下行室间隔缺损至肺动脉心内隧道补片方法修补和大动脉转换术方法纠治。其中1例冠状动脉畸形，结果：全组手术死亡1例，该例术后心功能不全，尿量少。余4例恢复良好，术后10－24d出院，结论：大动脉转换术治疗Taussig-Bing综合征，能防止术后左室流出道梗阻，避免了心外管道应用，减少了术后病死率，提高了术后远期疗效。     

大动脉调转术后吻合口梗阻的影响因素

目的 分析大动脉调转术后主动脉和肺动脉吻合口梗阻的影响因素.方法 1999年12月至2007年12月,行大动脉调转术(ASO)331例,术后生存288例.228例平均随访(20.4±18.6)个月,随访率79.2%.根据ASO术后超声报告所测主、肺动脉吻合口流速的大小,对完全性大血管错位室间隔完整型(TGA/IVS),完全性大血管错位伴窒间隔缺损(TGA/VSD),右室双出口伴肺动脉瓣下室间隔缺损、肺动脉高压(Taussig-Bing)和快速二期大动脉调转术(Stage-Switch)的随访资料分别分析其主、肺动脉吻合口的梗阻情况.对ASO手术后的各类疾病的主动脉和肺动脉吻合口流速,按流速<2 m/s,2～3 m/s,>3 m/s的病例百分数进行统计分析.结果 4种疾病分类的随访结果示主动脉吻合口流速差异有统计学意义(P=0.034),肺动脉吻合口流速差异无统计学意义(P>0.05).肺动脉吻合口流速增快发生率比主动脉吻合口高.Taussig-Bing组发生率高,Stage-Switch组发生率低.手术时病婴年龄越小(≤12 d),主动脉、肺动脉吻合口流速越易增快.随访时间延长,流速增快的发生率会逐渐提高.全组6例由于左、右流出道梗阻而再次手术.结论 ASO术后,肺动脉吻合口梗阻发生率较高.ASO术后必须定期随访,注意观测吻合口的生长情况.     

自体主动脉瓣移植纠治完全性大血管错位伴肺动脉瓣狭窄

目的探讨解剖纠治完全性大血管错位（TGA）伴肺动脉瓣狭窄的手术方法。方法1例8月龄、体重6．5kg的TGA伴肺动脉瓣狭窄男病婴，术中将原主动脉瓣移植至肺动脉瓣处形成新的主动脉；再行Switch术，同种带瓣管道连接右心室与肺动脉。另1例10月龄、体重9．8kg的该病男病婴，术中将整个主动脉瓣取下，保留左、右冠状动脉，向后移植，后半部分直接与原肺动脉瓣环连续缝合，前半部分与室间隔缺损之间采用dacron补片连续缝合关闭；肺动脉与右心室切口直接连接。结果2例手术均取得成功。出院时超声检查各吻合口通畅，无残余分流．无主动脉瓣反流。术后随访12个月和6个月，生长发育良好，心胸比率0．75。超声示左心功能良好，前例示主动脉瓣轻度反流．后例左、右心室流出道无残余梗阻．结论采用自体主动脉瓣移植纠治完全性大血管错位伴肺动脉瓣和瓣下狭窄，从解剖上得到彻底纠治．效果良好。由于病例少，随访时间短．还需进一步观察。     

右心室双出口的解剖矫治

目的 总结不同方法进行解剖矫治右心室双出口(DORV)经验.方法 2007年5月至2012年5月,收治135例DORV患儿,男79例,女56例;年龄为出生后25天～12岁;体质量3.5 ～ 30.0 kg.根据室间隔缺损的位置选择不同手术方法:89例主动脉瓣下缺损及33例双瓣下缺损患儿均行右心室双出口解剖矫治术;8例肺动脉瓣下缺损(Taussig-Bing畸形)患儿中5例行Rastelli手术,3例行动脉调转术±室间隔修补术;5例远离大动脉缺损中3例行Rastelli手术,2例行动脉调转术±室间隔修补术.结果 住院死亡5例,占3.70％;分别为1例肺动脉瓣下缺损行动脉调转术±室间隔修补术,1例双瓣下缺损,2例主动脉瓣下缺损(均合并肺动脉瓣狭窄)均行合右室双出口解剖矫治术,1例远离大动脉缺损行Rastelli手术者.结论 右室双出口病理解剖比较复杂,须根据不同的解剖条件选择不同的手术方法才能减少手术死亡.     

The Damus-Stansel-Kaye procedure: anatomical determinants and modifications

Seven of 119 patients undergoing anatomical correction for transposition of the great arteries and Taussig-Bing anomalies without pulmonary stenosis had the Damus-Stansel-Kaye procedure and the rest, the arterial switch. The age of the patients having the Damus-Stansel-Kaye procedure ranged from 0.5 year to 5 years (mean age, 2.2 +/- 1 years). Four patients had transposition, 2 had Taussig-Bing anomaly, and 1 had corrected transposition. Indications for the Damus-Stansel-Kaye procedure were side-by-side great arteries associated with difficult coronary anatomy (5 patients), single coronary system (1 patient), and subaortic stenosis (1). A graft between the ascending and descending aorta for interrupted aortic arch made mobilization and posterior displacement of the ascending aorta for the arterial switch difficult. Subaortic stenosis (1 patient), subpulmonary ventricular septal defect (2 patients), and restrictive ventricular septal defect (4) precluded the Rastelli procedure. In 6 patients, the main pulmonary artery was transected at the band, a proximal main pulmonary artery to aorta anastomosis was complemented with a synthetic patch, and a right ventricle to distal main pulmonary artery valved conduit was inserted. Four patients had closure of the aortic outflow. Two patients had postoperative bleeding and 2, heart block. The only patient who did not have transection of the main pulmonary artery, an omission that led to an obstructed conduit at the distal anastomosis, died late. Two patients subsequently needed aortic outflow closure for critical aortic insufficiency. The Damus-Stansel-Kaye procedure has a definite role and can be safely performed in patients with transposition of the great arteries and Taussig-Bing anomalies.(ABSTRACT TRUNCATED AT 250 WORDS)     

Anatomic correction for complete transposition and double-outlet right ventricle

Between February, 1981, and December, 1984, 30 patients underwent anatomic correction of transposition of the great arteries with intact ventricular septum (n = 8), transposition with ventricular septal defect (n = 15), and double-outlet right ventricle with subpulmonary ventricular septal defect, the Taussig-Bing anomaly (n = 7). At operation, ages ranged from 18 hours to 6 years (mean 11.3 months) and weights ranged from 2.6 to 16.4 kg (mean 6.1 kg). The group with transposition and intact ventricular septum on average was younger (mean 1.2 months) and smaller (mean 3.5 kg) than the other two groups. Associated congenital heart defects were seen in 12 patients, including five with coarctation, three with multiple ventricular septal defects, two with right ventricular hypoplasia, two with juxtaposed atrial appendages, and one each with interrupted aortic arch, Wolff-Parkinson-White syndrome, and left ventricular outflow tract obstruction. All 10 patients who had undergone prior palliative operations had pulmonary artery banding. In addition, four of these patients had coarctation repairs, four had atrial septectomy, and one had systemic/pulmonary shunting. All recognized patterns of coronary anatomy were encountered. The aorta and pulmonary artery were side by side in 14 patients and anteroposterior in 16 patients. The Lecompte maneuver to establish right ventricular-pulmonary arterial continuity was successfully used in 12 of 13 patients with anteroposterior great vessels but in none of those with side-by-side arteries. Seven patients had subvalvular right ventricular outflow tract obstruction, recognized either at operation (five) or postoperatively (two). This was responsible for death in three patients. The 30 day hospital mortalities were as follows: one death (12.5%) in the group with transposition and intact ventricular septum, six deaths (40%) in the group with transposition plus ventricular septal defect, and one death (14.3%) among patients with double-outlet right ventricle and subpulmonary ventricular septal defect; the overall mortality was eight deaths (26.7%). There have been no late deaths (mean follow-up 17.2 months). Ninety-five percent of the survivors are in New York Heart Association Functional Class I. Postoperative catheterization in 13 patients has shown normal left ventricular function, no coronary stenosis, and no aortic incompetence. Sixty-nine percent of these patients had clinically unsuspected gradients across the right ventricular outflow tract, which may be prevented by avoiding the Lecompte maneuver or the use of conduits.     

Surgical anatomy of the infundibular septum in transposition of the great arteries with ventricular septal defect

Anatomic variation of the infundibular septum was studied in transposition of the great arteries with ventricular septal defect in 23 hearts and double-outlet right ventricle with anterior position of the aorta in two hearts. Anterior displacement of the infundibular septum (i.e., "false" Taussig-Bing heart) was associated with coarctation or interruption of the aortic arch in 88% of the cases, whereas posterior displacement resulted in subpulmonary narrowing in 100% of the cases. Anterior displacement makes intraventricular rerouting from the left ventricle to the aorta difficult because of a long oblique route. In addition, the right ventricular cavity becomes smaller after closure of the ventricular septal defect. Therefore, arterial switch accompanied with transatrial or transpulmonary closure of the defect without ventriculotomy is recommended. In hearts with posterior displacement of the infundibular septum, the anterosuperior rim of the defect is difficult to approach through the tricuspid valve, and the route from the left ventricle to the aorta is rather straight. Hence, the Rastelli procedure is preferable. In hearts without displacement of the infundibular septum, either arterial or atrial switch with transatrial closure of the ventricular septal defect is applicable.     

心脏外科手术与介入性心导管术镶嵌治疗小儿先天性心脏病

目的 报道23例介入性心导管术与心脏外科手术镶嵌治疗小儿先天性心脏病(CHD)的经验。方法外科术后镶嵌治疗：12例动脉导管未闭结扎术后残余分流及2例心脏术后留置室间隔缺损(VSD)的复杂CHD行经导管封堵术。2例法洛四联症(TOF)根治术后分支肺动脉狭窄分别行球囊血管成形术及支架置入术，1例肺动脉闭锁合并VSD根治术后出现体肺侧支血管(APCAs)破裂行介入栓塞止血术。外科术前介入治疗：5例室间隔完整的大血管转位(TCA)病例行大动脉调转术前行球囊房隔造口术(BAS)，1例有巨大APCAs的重症。TOF在根治术前予以侧支血管堵塞术。结果动脉导管未闭术后残余分流及复杂CHD残留VSD病例均封堵成功，分支肺动脉狭窄病例介入治疗后狭窄减轻或治愈；侧支血管破裂病例栓塞止血后出血停止。BAS病例介入术后低氧及酸中毒改善，大动脉调转术后3例治愈，2例死亡。TOF病例APCAs堵塞后成功行根治术。结论心脏外科手术与介入性心导管术镶嵌治疗是治疗小儿CHD术后残余分流及部分复杂疑难CHD的有效方法。     

Arterial switch operation: factors impacting survival in the current era

Background. The arterial switch procedure has become the preferred procedure for the transposition of the great arteries (TGA) and Taussig-Bing anomaly. This analysis is intended to identify potential factors affecting survival in the current era.

Methods. From 1986 to 1999, 201 consecutive patients underwent an arterial switch operation for TGA or Taussig-Bing anomaly. Multivariate analysis of perioperative variables was performed for operative morbidity/mortality. Patients were separated into two groups. Phase 1 (n = 29) included patients before mid-1989 who underwent an open coronary reimplantation technique. Phase 2 (n = 172) included the patients undergoing a technique of reimplanting coronary buttons after neoaortic reconstruction.

Results. The patient population included TGA with intact ventricular septum (58.7%, 118 of 201), with ventricular septal defect (31.3%, 61 of 201), and Taussig-Bing anomaly (10.0%, 22 of 201). Overall, early mortality was 9.5% (19 of 201) and there were five late deaths (2.7%). One-month, 1-year, and 5-year actuarial survival rates were 90.4%, 87.9%, and 87.9%, respectively. Reoperation rate for late pulmonary stenosis was 2.7% (5 of 182). The freedom from reoperation at 3 and 5 years was 97.5% and 93.3%, respectively. In the analysis by time period, the operative mortality declined from 27.6% (8 of 29) to 6.4% (11 of 172) (p = 0.002). Risk factors for operative death were coronary artery patterns (usual vs retropulmonary left coronary artery, p = 0.009) in phase 1 and preoperative instability in phase 2 (p = 0.002).

Conclusions. The arterial switch operation for TGA and Taussig-Bing anomaly has early low and late mortality and reoperation rates. Technical modifications in coronary reimplantation have minimized coronary artery pattern-related risks.     

What is necessary for improvement of arterial switch operation?

We report long-term results of the arterial switch operation (ASO) and postoperative complications related to operative procedure. Between 1998 and 2007, ASO were performed in 42 patients [transposition of the great arteries (TGA) with intact ventricular septum:21, TGA with ventricular septal defect (VSD):13, Taussig-Bing anomary (TBA):7, and double outlet right ventricle with noncommitted VSD:1]. Hospital death occurred in 1 patient (2.4% mortality rate) due to low cardiac output syndrome (LOS) caused by prolonged aortic clamp, who had TGA with VSD, coactation of aortic arch and right ventricular outflow obstruction. Four patients required re-operation (freedom from re-operation rate was 84.7%). Two had pulmonary stenosis and 1 needed right ventricular outflow tract (RVOT) reconstruction with transannular patch. His pulmonary valve used for previous VSD closure was diminished. The other required muscle resection of RVOT. Five patients presented grade II or more neo-aortic insufficiency and 2 of them were TBA. Both these TBA, rerouting of left ventricular outflow tract (LVOT) to the aorta was challenging, they showed LVOT obstruction postoperatively. One underwent aortic valve plasty and the other had residual VSD closure. None had coronary event or abnormality in coronary arteriography. Selection of surgical procedure should be based on morphologic features in order to improve surgical outcome.