动脉转位术治疗65例新生儿完全性大动脉错位

目的报告美国西雅图儿童医院１９８９年１０月～１９９２年１０月，为完全性大动脉错位新生儿作动脉转位术６５例，其中伴室间隔完整型４１例，伴室间隔缺损型２４例。方法３６例使用深低温停循环方法，２９例使用深低温低流量方法。结果手术死亡４例（６．１５％），死亡原因均与冠状动脉解剖变异有关。结论动脉转位术是矫正新生儿期室间隔完整型和伴室间隔缺损型完全性大动脉错位最好的手术方法。     

大动脉转位术后吻合口生长的随访研究

目的随访分析大动脉转位术（ASO）后的主动脉（AO）、肺动脉（PA）吻合口生长情况,以了解ASO术后的长期疗效。方法回顾性分析自1999年12月至2007年12月上海交通大学医学院附属上海儿童医学中心施行ASO 331例患者的相关资料,其中完全性大动脉错位合并室间隔完整型（TGA／IVS）111例,完全性大动脉错位合并室间隔缺损（TGA／VSD）123例,右心室双出口伴肺动脉瓣下室间隔缺损、肺动脉高压（Taussig-Bing）73例,快速二期大动脉转位术（Stage-Switch）24例。随访228例,随访时间20．4±18．6个月;随访超声心动图报告752张,平均每例随访3．3次。根据超声心动图所测AO、PA吻合口直径大小,分析吻合口的生长情况。结果TGA／IVS患者AO、PA吻合口直径（近期为0．74±0．17cm和0．65±0．13cm,远期1．09±0．31cm和0．84±0．21cm）,TGA／VSD患者AO、PA吻合口直径（近期为0．76±0．20cm和0．63±0．14cm,远期为1．09±0．24cm和0．82±0．22cm）,Taussig-Bing患者AO、PA吻合口直径（近期为0．84±0．25cm和0．74±0．20cm,远期为1．05±0．30cm和0．85±0．24cm）远期较近期均有明显生长（P〈0．05）;Stage—Switch患者AO吻合口直径（近期为0．93±0．19cm,远期为1．19±0．29cm）远期相对于近期有明显生长（P〈0．05）,PA吻合口直径（近期为0．90±0．27cm,远期为1．00±0．32cm）远期较近期有生长,但差异无统计学意义（P〉0．05）。随访至2008年11月,共有6例患者因左、右心室流出道梗阻而需再次手术,术后有3例无残余梗阻,3例仍有残余梗阻。结论ASO的主动脉和肺动脉离断再缝合连接之后,血管能够随着年龄增加而生长,但也会发生狭窄。在长期随访中,有个别患者需要再次手术干预。     

快速二期大动脉转位术

完全性大动脉错位（D-TGA）约占先天性心脏病（先心病）的5％，是新生儿期常见、易发生心力衰竭、病死率最高的紫绀型先心病。病婴的主动脉起自右心室，肺动脉起自左心室，体肺循环成并列循环状态，肺循环的氧合血不能有效地进入体循环。病婴出生后，因体循环血氧饱和度过低，发生缺氧、酸中毒死亡。文献报道，若未及时行外科治疗，完全性大动脉错位室间隔完整（TGA-IVS）病婴1个月内的病死率为50％，90％的病婴将在1岁以内死亡。大动脉转位术（ASO）是纠治的最佳手术方案。[第一段]     

影响动脉转位术手术死亡的危险因素分析

目的探讨动脉转位术(arterial switch operation,ASO)手术死亡的危险因素,以提高手术成功率,降低手术死亡率。方法2001年1月1日至2007年12月31日期间在阜外心血管病医院行ASO208例,男157例,女51例;年龄6 h至17岁,中位年龄90 d;体重3～43 kg,中位体重5 kg。大动脉转位(TGA)/室间隔缺损(VSD)127例,TGA/室间隔完整(IVS)或合并VSD直径5 mm81例。冠状动脉分布正常(1LCx2R)151例,单冠状动脉畸形15例,壁内走行6例,冠状动脉分布反位36例。收集所有患者术前、术中和术后的临床资料,建立数据库,行单因素和logistic多因素分析影响ASO手术死亡的危险因素。结果围术期死亡24例(11.54%),死于术后感染合并多器官功能衰竭12例,低心排血量综合征10例,肺动脉高压危象1例,脑部并发症1例。其中2001～2005年手术死亡20例(18.30%),2006～2007年手术死亡4例(4.00%),后者手术死亡率较前者明显降低(P0.05)。单因素分析结果:死亡患者体外循环时间明显长于生存患者(236±93 min vs.198±50 min,P=0.002),冠状动脉分布异常(33.3%vs.6.5%,P=0.000)及术中发生冠状动脉事件(33.3%vs.2.2%,P=0.000)的比率明显高于生存患者;logistic回归多因素分析结果:手术年代早(OR=7.463,P=0.003)、冠状动脉分布异常(OR=6.303,P=0.005)及术中发生冠状动脉事件(OR=17.312,P=0.000)是ASO围术期死亡的主要危险因子。结论ASO有明显的学习曲线,手术年代早、冠状动脉分布异常、术中对冠状动脉处理不当是影响ASO死亡的主要危险因子。     

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     

大动脉转位术的术后处理

采用大动脉转位术（ａｒｔｅｒｉａｌｓｗｉｔｃｈｏｐｅｒａｔｉｏｎ，ＡＳＯ）治疗完全性大动脉转位（ＴＧＡ）。ＡＳＯ从解剖上完全纠正ＴＧＡ，效果良好。除手术技术，体外循环管理外，术后处理是降低死亡率，提高治疗效果的重要环节之一。自１９８７年９月～１９９２...     

利用“多余”冠状动脉纽片作主动脉根部成形在大动脉转位术中的应用

目的总结大动脉转位术(ASO)中利用"多余"的冠状动脉纽片作新主动脉根部成形的临床经验,探讨保持主动脉瓣窦的解剖形态对改善术后冠状动脉血流的临床意义。方法选取2003年1月至2009年6月,我院收治的室间隔缺损型大血管错位和Taussig-Bing畸形患者110例,手术年龄为出生2d～2岁,平均年龄91.1d;体重1.79～9.50kg,平均体重4.70kg。根据不同的外科处理技术,将患者分为两组,A组:78例,术中采用多余的冠状动脉纽片作新的主动脉根部成形,减小新主动脉根部近心端开口的直径;B组:32例,术中剪除多余的冠状动脉纽片,主动脉吻合口近心端与远心端直径不匹配。采用logistic逐步回归筛选结果中影响住院死亡的危险因素。结果术后早期死亡12例,总病死率为10.9%(12/110),A组病死率明显低于B组[6.4%(5/78)vs.21.9%(7/32),P=0.019]。随访72例,随访时间为术后3个月～5年。后期死亡3例,A组1例,B组2例;5例患者再次手术。单因素logistic回归分析结果显示:影响术后早期死亡的危险因子包括Taussig-Bing畸形(χ2=4.011,P=0.046)、合并主动脉弓病变(χ2=4.437,P=0.036)、单支冠状动脉(χ2=5.071,P=0.025)和B组患者(χ2=5.584,P=0.019)。多因素logistic回归分析结果显示:合并主动脉弓病变(χ2=5.681,P=0.010)和B组患者(χ2=3.987,P=0.047)是构成影响术后早期死亡的独立危险因子。结论利用"多余"的冠状动脉纽片作新的主动脉根部成形能够较好地保持新主动脉瓣窦形态,而主动脉根部的特殊解剖形态与冠状动脉灌注有重要的关系,手术病死率降低可能与术后冠状动脉灌注得到改善有关。     

完全性大动脉错位中的冠状动脉畸形分类及其处理

完全性大动脉错位是一种常见的紫绀型先天性心脏畸形，如不治疗婴儿早期即死亡。目前临床上对无肺动脉瓣狭窄的完全性大动脉错位患者采用动脉转位术纠治，但如合并冠状动脉畸形，手术的难度和死亡率就会增高。因此，处理好合并的冠状动脉畸形是手术成功的关键。现就冠状动脉畸形的分类、手术方法及处理结果进行综述。     

完全性大动脉转位手术的围术期管理

大动脉转位(transposition of great arteries,TGA)是指主动脉和肺动脉与心室的衔接异常,分为两型:完全性大动脉转位(图1A)和矫正性大动脉转位(图1B)。前者的发病率约占先天性心脏病的8％～10％,仅次于法乐四联症(12％～14％),是2个月内婴儿发生充血性心力衰竭的主要原因,若不及时治疗90％的患儿在1岁内死亡。后者血液循环已经达到生理上的矫正,若无合并其他畸形,不需要手术治疗。     

完全性大动脉错位的解剖矫正术

解剖矫正术治疗完全性大动脉错位（ＴＧＡ）８例，其中４例伴室缺（ＶＳＤ），４例室隔完整（ＩＶＳ）。除２例年龄分别为３岁和７岁外，其余均小于６个月；其中４例平均年龄３０天，体重３．６ｋｇ。４例ＴＧＡ／ＩＶＳ无医院死亡，４例ＴＧＡ／ＶＳＤ死亡１例，总死亡率１２．５％。随访３月～３年，手术效果满意。我们认为新生儿室隔完整型ＴＧＡ和ＴＧＡ伴室缺者，采用解剖矫正术较心房内血流转换术为好。     

双活瓣延长技术在复杂动脉转位术中的应用

目的总结在动脉转位术(arterial switch operation,ASO)中采用双活瓣延长技术做冠状动脉移植治疗完全型大动脉转位(D-transposition of the great arteries,D-TGA)和Taussig-Bing畸形的临床经验,探讨该技术的手术方法和手术适应证。方法回顾性分析2006年1月至2011年6月上海交通大学医学院附属上海儿童医学中心21例合并复杂冠状动脉畸形的D-TGA、L-TGA或Taussig-Bing患者[男13例,女8例;年龄(110.0±84.5)d;体重(5.4±4.2)kg]行ASO治疗,术中采用双活瓣延长技术做冠状动脉移植的临床资料。所有患者均为右冠状动脉主干或粗大右心室圆锥支发自主动脉左侧或右侧瓣窦,并异常前绕于主动脉根部行走。双活瓣延长技术包括:从主动脉剪取较长的冠状动脉纽片做为活瓣;剪取肺动脉(新主动脉)另一带蒂活瓣向冠状动脉纽片方向做等距离延长;活瓣与纽片的边缘互相缝合形成冠状动脉的延长管道。结果无住院死亡,术后呼吸机使用时间(101.6±53.6)h,监护时间(9.5±4.9)d。术后发生低心排血量9例,肺动脉高压危象2例,肺部感染6例,急性肾功能衰竭2例,均经相应的治疗治愈出院。11例患者术后延迟关胸。随访17例,随访时间2个月～5年,随访期间患者生长发育均明显改善,无缺血性心电图改变。1例肺动脉瓣上狭窄患者于ASO术后2年再次行手术修补。结论双活瓣延长技术做冠状动脉移植在复杂ASO中能有效减少因冠状动脉畸形导致的手术死亡,尤其适合于二期ASO以及右冠状动脉主干或粗大圆锥支发自左侧或右侧瓣窦,并沿主动脉异常前绕的患者。     

Anatomical correction of complex forms of transposition of the great arteries in neonates

Objective—The arterial switch operation has become the procedure of choice for the simple transposition of the great arteries (TGA) while in the complex forms of the defect the staged approach is frequently implemented. The aim of this study is to compare groups of patients with simple transposition and children with the complex form of the defect and identify factors affecting early and late outcome.

Design—From 1997 to 2003, 135 consecutive neonates with TGA underwent arterial switch operation and simultaneous reparation of all associated defects. Univariate and multivariate analysis of perioperative variables and follow‐up data was performed. Patients were divided into two groups. Group I (n=84, 62.2%) included neonates with simple transposition (TGA/IVS), Group II (n=51, 37.8%) included children with complex transposition (TGA/VSD).

Results—Overall early mortality was 8.1% and there was one late death (0.7%). One‐month, 1‐year and 5‐year actuarial survival rates were 91.8, 91.1 and 91.1%, respectively. There were no differences in the early and late survival rate between groups. Reintervention rate for right ventricular outflow tract obstruction (RVOTO) was 13.3% (balloon plasty or reoperation). The freedom from reintervention at 1, 3 and 5 years was 98.4, 87.9 and 85.4%, respectively. There were no differences in the need for reintervention between groups. The significant differences between groups concerned: age at operation (p?<?0.001), associated anomalies (p=0.002) including aortic arch anomalies (p=0.002) and coronary artery anomalies (p=0.02), application of delayed chest closure (p=0.015), and occurrence of sepsis (p=0.032). Risk factors for early death were: left ventricule dysfunction related to age at operation (p=0.016) and resternotomy in intensive care unit (p?<?0.001). There were no differences between groups concerning these risk factors as far as circulatory arrest time, aorta clamping time, and early and late morbidity.

Conclusions—The arterial switch operation can be the treatment of choice for various forms of TGA with low early and late mortality and morbidity rates. The main cause of early death is still left ventricular dysfunction. Such well‐known predictors of poor outcome as presence of ventricular septal defect, coronary artery anomalies and aortic arch anomalies did not affect early and late findings. The presented approach of early simultaneous anatomical correction of TGA and all associated anomalies ensures good condition of children with low necessity for reintervention.     

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。     

Long-term coronary artery outcome after arterial switch operation for transposition of the great arteries

Objective: To analyse the long-term patency of coronary arteries after neonatal arterial switch operation (ASO). Methods: A retrospective study of the operative reports, follow-up and postoperative catheterisation data of 119 patients, who underwent the great arteries (TGA) repair since 1991, has been carried out. Patient population: Among the 133 survivors of the 137 ASOs performed between 1991 and 2007, 119 patients have been studied by routine control cardiac catheterisation and form the study population. Median time between repair and the coronary angiography was 2.9 ± 1.9 years. A comparison between the eight patients (6.7% out of the entire study population), known to have postoperative coronary obstructions (group I) and the rest of the cohort with angiographic normal coronary vessels (group II) was performed by univariate analysis of variance and logistic regression models. One patient had surgical plasty of the left coronary main stem with subsequent percutaneous angioplasty, three patients had primary coronary stent implantation and four patients had no further intervention at all. In group I, all but one patient denied symptoms of chest pain and echocardiography failed to show any difference between the two groups in terms of left ventricular systolic function (ejection fraction group I 61 ± 2% vs 62 ± 6% of group II, p = 1.0). Results: The association of coronary obstruction with complex native coronary anatomy (Yacoub type B to E) was evident at both univariate (62% of group I vs 22% of group II, p = 0.04) and logistic regression (p = 0.007, odds ratio (OR) 8.1) models. The type of coronary reimplantation (i.e., coronary buttons on punch vs trap-door techniques) was similar between the two groups (punch reimplantation in 25% of patients of group I vs 31% of group II, p = 0.1) as was the relative position of the great vessels (aorta anterior in 100% of patients of group I vs 96% of group II; univariate, p = 0.1). Conclusions: The late outcome in terms of survival and functional status after ASO is excellent. Nevertheless, the risk of a clinically silent late coronary artery obstruction of the reimplanted coronary arteries warrants a prolonged follow-up protocol involving invasive angiographic assessment.     

Early and long term outcome of the arterial switch operation for transposition of the great arteries: predictors and functional evaluation

Objectives: The aims of this report were to study the early and late outcome in terms of mortality, freedom from reoperation, predictors for late pulmonary stenosis (PAS) and insufficiency of the neo-aortic valve (AVI) in patients with transposition of the great arteries (TGA) undergoing arterial switch operation (ASO). Materials and methods: Between January 1990 and December 2001, 134 patients with TGA underwent ASO. The patients were divided in Group I (n=88)-TGA with intact ventricular septum and Group II (n=46)-TGA with ventricular septal defect (VSD). The pulmonary artery was reconstructed employing the direct anastomosis technique (PT-I) in 21 (15.7%) patients, the double-patch technique (PT-II) in 41 (30.6%), single pantaloon patch (partial circumference) (PT-III) in 46 (34%) and single pantaloon patch (total circumference) (PT-IV) in 35 (26%) patients. The mean follow-up was 3.4±1.3 years. Results: The hospital mortality was 17 (12.7%) patients. The mortality in Group I was significantly lower than Group II (P=0.002). The overall actuarial survival at 1, 3 and 5 years follow-up resulted to be 98, 93, and 91.5%, resulting to be significantly higher in Group I (P=0.032). The multivariate analysis revealed the complex TGA (P=0.007), VSD (P=0.032), coronary anomalies (P=0.004), aortic coarctation or hypoplastic aortic arch (P=0.021), left ventricular outflow tract obstruction (LVOTO) or moderate PAS (P=0.041) as strong predictors for poor free-reoperation cumulative survival. A strong inverse correlation was found between the mean trans-pulmonary gradient at follow-up and the age at the operation (r=−0.41, P<0.0001). The univariate analysis revealed the PT-I technique (P=0.002), prior moderate PAS (P=0.0001), and age <1 month (P=0.018) as strong predictors for moderate-to-severe PAS. The neo-AVI incidence was significantly higher in Group II (P=0.011). Predictors for neo-AVI were male sex (P=0.003), preoperative neo-AV Z-score >1 (P<0.001), prior or concomitant operation for aortic coarctation or hypoplastic aortic arch (P=0.001), LV retraining (P=0.003). Conclusion: ASO remains the procedure of choice for the treatment of various forms of TGA with acceptable early and later outcome in terms of overall survival and free reoperation. Strong predictors for poor overall free-reoperation survival are complex TGA, VSD, coronary anomalies, aortic coarctation and LVOTO or moderate PAS. The pulmonary artery reconstruction using a single ‘pantaloon patch’ seems to offer less residual stenosis. Patients with a VSD and a significant mismatch between the neo-aortic root and distal aorta are at a higher risk for developing postoperative neo-AVI.