大动脉转换术在复杂先天性心脏病治疗中的应用

目的 回顾总结我院近3年采用大动脉转换术治疗小儿复杂先天性心脏病(先心病)的临床经验。方法2000年1月至2003年5月,采用大动脉转换术(Switch手术)治疗小儿复杂先心病61例。其中完全性大血管错位(TGA)45例,包括室间隔完整型(TFGA-IVS)26例,伴室间隔缺损(TGA,VSD)19例;右心室双出口伴肺动脉瓣下室间隔缺损(Taussig-Bing)16例。结果TGA,IVS死亡2例;TGA-VSD死亡4例;Taussig-Bing死亡4例;总手术病死率16％。术后随访3个月-3年,所有病例青紫症状消失,活动能力明显增强。1例Taussig-Bing术前二尖瓣轻～中度返流,术后仍为中度返流。2例TGA术后出现主动脉和肺动脉瓣上狭窄,压差40mmHg,1例肺动脉瓣下狭窄和残余室间隔缺损,3个月后再次手术治愈。结论大动脉转换术应用于完全性大血管错位的纠治,手术效果满意;应用于右室双出口肺动脉瓣下室间隔缺损的纠治,不但可早期纠治,防止其肺血管阻塞性病变发生,而且避免了心内修补左室流出道梗阻的远期并发症。     

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。     

心房内调转术治疗完全性大动脉错位

目的总结用心房内调转术治疗完全性大动脉错位(TGA)患者的经验。方法自2002年1月至2005年3月共对10例TGA患者行心房内调转术,男6例,女4例,年龄3个月~12岁。TGA伴室间隔缺损、肺动脉高压7例,伴房间隔缺损、室间隔完整2例,伴室间隔缺损、动脉导管未闭1例。采用Senn ing手术4例,M ustard手术6例。结果10例患者无1例死亡,脉搏血氧饱和度由手术前的0.65±0.05增至术后的0.95±0.03。紫绀消失,体质增强。随访4~28个月,复查心脏彩色超声心动图提示心房内转流正常,无梗阻现象,三尖瓣无明显反流。结论对TGA患者施行心房内调转术,可以取得较好的临床效果。     

主动脉移位术纠治婴幼儿完全性大动脉错位伴室间隔缺损和肺动脉狭窄

目的探讨主动脉移位术纠治完全性大动脉错位伴室间隔缺损和肺动脉狭窄(TGA/VSD/PS)的临床应用。方法2004年8月到2005年7月,采用主动脉移位术连续纠治6例TGA/VSD/PS,其中男5例,女1例;年龄4~24个月,平均(11.33±6.86)个月;体重5.6~11.0kg,平均(8.43±2.19)kg。结果无手术死亡。体外循环转流112~204min,平均(153.83±33.70)min,主动脉阻断73~139min,平均(89.16±18.61)min。术后随访2~12个月。6例病儿活动良好,X线胸片示心影较术前略大,肺血增多;心电图示窦性心律,心脏超声检查示主动脉瓣反流轻微2例,轻度1例。射血分数0.74~0.86,短轴缩短率0.34~0.52。结论当前TGA/VSD/PS普遍采用Rastelli手术,但长期随访发现术后并发症较多,结果尚不理想。主动脉移位术重建左心室流出道和右心室流出道,避免了Rastelli手术后左心室流出道梗阻和心外管道梗阻的并发症。     

一期手术矫治主动脉弓中断及伴发畸形

目的总结主动脉弓中断(IAA)及伴发畸形的手术治疗经验。方法自2000年1月至2005年12月我科共收治48例IAA及伴发畸形患者(平均年龄1.1岁、平均体重7.0kg),合并畸形有室间隔缺损23例,完全性大动脉错位2例,主-肺动脉窗伴右肺动脉起源于主动脉3例,第5主动脉弓残存狭窄2例,右心室双出口2例,永存动脉干2例,迷走右锁骨下动脉伴降主动脉发育不良1例。48例中35例接受手术纠治,其中34例IAA及伴发畸形一期解剖纠治,1例伴右心室双出口患者行主动脉弓吻合及肺动脉环缩术纠治。结果手术死亡4例。术后26例随访3个月~4年,死亡2例。术后发生并发症7例,Ⅲ°房室传导阻滞和膈神经麻痹各1例,轻度吻合口梗阻5例,其余患者无残余解剖问题,心功能良好。结论在新生儿期施行IAA一期矫治手术成功率高,围术期合适的干预是治疗成功的关键;由于伴发畸形复杂,远期生存率不容乐观。     

先天性主动脉弓中断及合并畸形的一期手术治疗

目的回顾总结先天性主动脉弓中断及合并畸形一期手术治疗经验。方法1988年7月至2004年8月,手术治疗主动脉弓中断及合并畸形20例。男12例,女8例;手术年龄18d～9岁。其中<1岁者10例,平均年龄(3.37±2.45)个月,平均体重(5.33±1.72)kg;>1岁者10例,平均年龄(2.60±1.24)岁,平均体重(13.10±6.53)kg。所有病儿术前均有反复呼吸道感染史。A型15例,B型5例。2例经双切口手术,即先通过左后外侧切口纠治主动脉弓中断,再经正中切口纠治合并畸形。18例采用正中切口同时纠治主动脉弓中断和合并畸形。结果全组死亡3例,死于术后严重肺动脉高压危象2例、严重心功能衰竭1例。17例随访3个月～12年,均恢复良好。结论主动脉弓中断采用正中切口方法,操作简便,暴露清楚,创伤小,有利于术后恢复。本病早期即出现肺动脉高压,临床上一经确诊宜尽早手术。     

室间隔完整型大动脉错位的手术治疗

目的　探讨室间隔完整型大动脉错位 (TGA/IVS)的手术治疗方法。　方法　回顾性总结 1999年 8月～ 2 0 0 1年 12月 ,手术纠治TGA/IVS 12例的临床资料。 3例年龄 >2个月的患儿行Senning手术 ,9例年龄 <1个月的采用大动脉转换术 (switch)。　结果　本组采用switch手术治疗者 1例死亡 ,死亡原因为单根冠状动脉。 11例存活患者 ,随访 2个月～ 2年 ,平均 (12± 7)个月 ,效果良好。结论　室间隔完整型大动脉错位必须早期做出诊断 ,以便及时得到治疗。switch手术的最佳手术年龄应在婴儿 2周内 ,最好不超过 1个月     

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。     

室间隔完整的肺动脉闭锁外科治疗

目的探讨适当的手术方式,评价手术矫治室间隔完整的肺动脉闭锁的临床疗效。方法1992年1月至2004年8月手术治疗17例室间隔完整的肺动脉闭锁病儿,男9例,女8例;年龄15d~12岁,平均(25.5±7.9)个月;体重3.5~28.0kg,平均(7.8±5.4)kg。其中根治手术10例,三尖瓣Z值-2.3~1.2,平均(-0.78±0.34),肺动脉发育指数149.53~297.89mm2/m2,平均(206.35±82.15)mm2/m2,其中2例病儿因严重低氧血症术后次日加行体肺分流手术;生理矫治手术6例,Z值-6.1~0.2,平均(-2.7±0.92),肺动脉发育指数39.88~218.29mm2/m2,平均(131.85±72.93)mm2/m2,包括双向格林手术2例,体肺动脉分流手术1例,右室流出道重建合并体肺动脉分流术3例;一个半心室矫治术1例,首先行双向格林手术,2年后行房间隔缺损修补术,动脉导管结扎术和右室流出道重建术。结果围术期死亡3例(16.67%),2例为先行根治术次日加行体肺分流手术者,1例为右室流出道重建合并体肺动脉分流术者。术后并发症低心排综合征3例,低氧血症3例,乳糜胸1例,右心衰3例,余者均顺利康复。结论合并室间隔完整的肺动脉闭锁是一种少见的先天性心脏病,依照右心室大小、三尖瓣环大小、肺动脉发育情况及冠状动脉解剖等采用适当的手术方式可取得满意的治疗效果。     

上腹部手术史患者行腹腔镜胆囊切除术的临床体会

目的:探讨有上腹部手术史患者行腹腔镜胆囊切除术(laparoscop ic cholecystectomy,LC)的可行性及要点。方法:回顾分析2004年10月至2010年12月为128例有上腹部手术史的患者施行LC的临床资料。结果:115例成功完成LC,13例中转开腹,成功率89.84%。LC组手术时间35~240m in,平均(67.7±19.4)m in;中转组73~225m in,平均(89.3±16.8)m in。LC组术中出血量5~100m l,平均(23.7±10.6)m l;中转组50~1 000m l,平均(200.6±45.9)m。lLC组术后住院3~5d,平均(3.6±0.8)d;中转组8~23d,平均(11.3±3.4)d。LC组术后无并发症发生;中转开腹组1例切口脂肪液化,1例切口感染,无其他并发症发生。所有病例均治愈出院。结论:有上腹部手术史不应成为LC的禁忌,但具体术式应根据术前判断和患者的具体情况决定。     

Taussig-Bing anomaly and arterial switch: aortic arch obstruction does not influence outcome

OBJECTIVE: Aortic arch obstruction is a commonly associated problem in the Taussig-Bing anomaly. Between 1983 and 1995, 28 consecutive patients with Taussig-Bing anomaly underwent arterial switch operation with baffling of the left ventricle to neoaorta. Group A: 11/28 had associated aortic arch obstruction. Group B: 17/28 had isolated Taussig-Bing anomaly. We assessed whether the coexistence of subpulmonary ventricular septal defect and aortic arch obstruction pose an incremental risk factor. PATIENTS AND RESULTS: Group A: Mean age and weight were 1.4+/-1.3 months and 3.5+/-0.4 kg. The aortic arch obstruction included: hypoplasia (5/11), interruption (4/11) and discrete coarctation (2/11). Seven patients had a one-stage correction, and four had initial arch repair followed by arterial switch operation. There were no hospital deaths (CL 0-28%). Over a follow-up of 638 patient-months (mean 64+/-39), there have been no late deaths, and all patients are in New York Heart Association class 1. There have been three cases of recurrent aortic arch obstruction (two requiring reoperation, and one requiring balloon dilation). One patient has been reoperated upon for right ventricular outflow tract obstruction. The actuarial survival and freedom from reoperation rates at 6 years were 100% (CL = 66-100%) and 72.9% (CL=38-92%) respectively. Group B: Mean age and weight were 5.9+/-8.4 months and 5+/-2.1 kg. All patients had a one-stage operation. There were two early deaths (11.8%, CL = 1-36%) and one late death over a follow-up of 678 patient-months (mean 52+/-31). All survivors are in New York Heart Association class 1 and there have been no reoperations. The actuarial survival and freedom from reoperation rates at 6 years were 81% (CL = 56-93%) and 100% (CL = 76-100%) respectively. CONCLUSIONS: 1. Aortic arch obstruction has not adversely affected early or late survival (P>.05) or late functional class. 2. Patients with Taussig-Bing anomaly and aortic arch obstruction may have a higher reoperation rate than those with normal arch anatomy. 3. Taussing-Bing anomaly, with or without aortic arch obstruction, can be repaired with arterial switch operation during the neonatal period with good outcome.     

Results of arterial switch operation for primary total correction of the Taussig-Bing anomaly

BACKGROUND: This study evaluates the results of the arterial switch operation for early total repair of double-outlet right ventricle with subpulmonary ventricular septal defect (the Taussig-Bing heart). METHODS: From 1986 through April 2003, 27 patients with Taussig-Bing anomaly underwent arterial switch operation. Twenty patients were neonates (n = 11) or infants younger than 3 months (n = 9). Obstruction of aortic arch (n = 19) or subaortic right ventricular outflow tract obstruction (n = 20) and unusual coronary artery patterns (n = 19) were common. Total correction as a single procedure was performed in 21 patients. Events are depicted by Kaplan-Meier curves. RESULTS: There was 1 patient hospital death at 2 months after repair. One patient died late that was not cardiac related. Survival was 92% +/- 6% at 8 months and remained constant thereafter. Four patients underwent reoperation (1 for residual aortic arch obstruction and 3 for subvalvular and valvular pulmonary stenosis). Freedom from reoperation decreased to stabilize at 83% +/- 8% after 2 years. The risk to have right ventricular outflow tract obstruction develop was 33% +/- 10% at 1 year, increasing slowly and leveling out at 57% +/- 12% at year 5 and thereafter. Statistical analysis revealed no significant risk factor for death or need for reoperation. CONCLUSIONS: The Taussig-Bing anomaly should be corrected in the neonatal period or in early infancy by arterial switch operation, closure of the ventricular septal defect, and simultaneous correction of associated cardiovascular anomalies as a one-stage procedure. Right ventricular outflow tract obstruction often complicates the postoperative course and is the main cause for reintervention.     

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。     

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.     

主动脉弓中断的外科治疗

目的 总结主动脉弓中断及合并心脏畸形的外科治疗经验.方法 1997年1月至2008年1月,36例主动脉弓中断患者进行了外科手术治疗,其中男性22例,女性14例.儿童患者36例,年龄2个月～7岁,平均年龄2.8岁.成人患者1例,年龄31岁.33例合并心内畸形,其中31例正中开胸同时矫治主动脉弓中断和心内畸形;1例左侧切口矫治主动脉弓中断,正中开胸修补心内畸形;1例采用姑息手术.3例无心内畸形的患者2例采用左后外侧切口,1例采用正中胸部加上腹部切口.术式包括16例管道连接,9例直接吻合,9例直接吻合并补片成形,1例应用左锁骨下动脉翻转.在31例正中切口一期手术中,17例应用选择性脑灌注加下半身停循环,8例采用深低温低流量灌注,6例采用全身停循环.结果 住院死亡5例,3例死于肺部感染,1例死于肺动脉高压危象,1例死于术后低心排血量.术后早期有其他重要并发症7例.31例存活患者随访3个月～5年,无远期死亡,无需要再次手术的病例.结论 合并心内畸形的主动脉弓中断患者可采取选择性脑灌注加下半身停循环或深低温全身低流量下正中一期手术同时矫治.     

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.     

自体肺动脉补片主动脉弓成形治疗婴儿主动脉缩窄合并弓发育不良

目的 探讨缩窄段切除加自体肺动脉补片主动脉弓成形治疗婴儿主动脉缩窄合并主动脉弓发育不良的方法和效果.方法 2007年5月至2009年12月,14例主动脉缩窄合并主动脉弓发育不良病婴行主动脉缩窄段切除加自体肺动脉补片主动脉弓成形手术,其中男9例,女5例;年龄23天至17个月,中位值4.33月龄;平均体重(6.14±2.36)kg.所有病婴均诊断为合并室间隔缺损的主动脉缩窄,同时存在主动脉弓发育不良.手术在深低温体外循环下完成,其中8例采用选择性脑灌注技术,6例停循环.主动脉成形采用新鲜的自体肺动脉补片.同期修补合并的室间隔缺损.结果 围术期死于循环衰竭1例.13例生存,其中1例合并低心排血量综合征,经相应治疗恢复.术后超声心动图检查主动脉弓无残余梗阻.随访4个月至3年.超声心动图示主动脉弓压力阶差均<16 mm Hg(1 mm Hg=0.133 kPa),随访期间主动脉弓降部血流速度与出院时无明显变化.CT扫描显示主动脉弓几何构形正常;术后半年原左主支气管受压者症状明显改善或完全消失.无主动脉夹层动脉瘤发生.结论 缩窄段切除加自体肺动脉补片主动脉弓成形是治疗婴儿主动脉缩窄合并主动脉弓发育不良理想的手术方法.

Abstract：

Objective To discuss the operative techniques and results of coarctation resection plus aortoplasty with pulmonary autograft patch for coarctation of the aorta combined with hypoplastic aortic arch in infant.Methods Between May 2007 and Dec 2009,14 cases including 9 males and 5 females with caorctation of the aorta and hypoplastic aortic arch underwent coarctation resection plus aortoplasty with pulmonary autograft patch in our hospital.The age ranged from 23 days to 17 months,with a median of 4.33 months.The mean body weight was (6.14 ±2.36) kg.All patients were diagnosed as aortic coarctation combined with VSD and hypoplastic aortic arch.The surgery was performed under deep hypothermia cardiopulmonary bypass with selective cerebral perfusion in 8 cases and circulation arrest in 6 cases.Fresh pulmonary autograft patch harvested from the main pulmonary artery was used for aortoplasty.The associated VSD was repaired in the same stage.Results All patients survived except one died from circulatory failure during the perioperative period.Low cardiac output syndrome occurred in another case who was cured afterwards by correspondent treatments.No residual obstruction was detected by echocar-diography after the operation.Follow-up was carried out in 13 cases from 4 months to 3 years.Echocardiographic examination showed that the pressure gradient across the aortic arch was less than 16 mm Hg in all cases.The blood velocity at the descending aortic arch was not significantly changed during the follow-up period as compared with that of the immediate after operation.Computed tomography showed that the morphology of aortic arch was normal.The left bronchus compression was relieved obviously or totally disappeared in patients who suffered from left bronchus stenosis before operation,and no aortic aneurysm were detected in these patients.Conclusion Conclusion Coarctation resection plus aortoplasty with pulmonary autograft patch is the optimal surgical method for treating coarctation of the aorta combined with hypoplastic aortic arch in infant.     

主动脉缩窄或主动脉弓中断合并心内畸形一期修复

目的 探讨采用经胸骨正中切口一期修复主动脉缩窄(CoA)或主动脉弓中断(IAA)合并心内畸形的治疗效果.方法 2002年7月至2009年6月,经胸骨正中切口行降主动脉远端和主动脉弓下缘端侧吻合术一期修复CoA或IAA合并心内畸形病儿43例,其中CoA 34例,IAA 9例(A型6例、B型3例),合并心内畸形包括室间隔缺损42例、动脉导管未闭34例、房间隔缺损12例、主动脉瓣下隔膜狭窄5例、二尖瓣关闭不全2例,右心室双出口1例.结果 手术死亡1例,为术后肺动脉高压和严重低心排血量综合征者.术后并发症包括严重低心排血量综合征3例,低氧血症6例,肺部炎症11例,肺不张14例,声音嘶哑19例,室上性心动过速23例.8例失访.34例随访3个月～5年,生活质量明显改善,心脏超声心动图和CT检查显示吻合口无明显再缩窄发生.结论 经胸骨正中切口,采用主动脉远端和主动脉弓下缘端侧吻合技术一期修复CoA或IAA合并心内畸形的手术早、中期效果良好,能明显减少术后再狭窄.