Nikaidoh术治疗完全型大动脉错位伴室间隔缺损和肺动脉狭窄的早期结果

目的回顾性研究Nikaidoh术治疗完全型大动脉错位伴室间隔缺损和肺动脉狭窄(TGA/VSD/PS)的早期结果。方法在2004年1月至2005年12月期间,有8例TGA/VSD/PS患者在我院接受Nikaidoh术矫治,手术年龄4～29个月(11.4±7.6个月),体重5.2～11.0kg(8.0±1.9kg);所有患者房室连接一致,其中伴房室瓣骑跨1例,左肺动脉狭窄1例,本组患者术前均未行其他手术,手术均采用改良Nikaidoh术,即主动脉根部移位和重建左、右心室流出道,除1例冠状动脉同时移植和使用Homograft重建右心室流出道外,用自体心包补片扩大重建右心室流出道。结果手术死亡1例,无1例出现明显左室流出道梗阻(LVOTO)和右室流出道梗阻(RVOTO),轻度肺动脉反流3例,中度4例,除1例死亡患者外,其余左心功能均正常;随访时间平均8.8个月(3～18个月),7例存活;心功能状况佳,射血分数(EF)0.64±0.02;短轴缩短率(FS)0.33±0.02。未出现进展性主动脉瓣反流、LVOTO、RVOTO和肺动脉反流加重。结论Nikaidoh术适宜于治疗TGA/VSD/PS,尤其当解剖上存在不宜行Rastelli术的患者,早期结果良好。     

改良Nikaidoh手术治疗伴左室流出道狭窄的大动脉转位

2004年2月至10月，我们在Nikaidoh手术基础上，用改良术式治疗2例大动脉转位（TGA）合并室间隔缺损（VSD）和肺动脉狭窄（PS）的病儿，以解决术后冠状动脉供血不足和重建后右室流出道非生长性的难题，手术取得了很好的近期疗效，现报道如下。     

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。     

Nikaidoh手术的临床应用

目的 总结Nikaidoh手术治疗右心室双出口(DORV)和大动脉转位(TGA)患者的临床经验,以提高手术疗效. 方法 3例先天性心脏病患者中DORV 1例,TGA 2例(平均年龄11.6岁),均行Nikaidoh手术,术中行Lecompte操作,Gore-tex人工血管补片修补室间隔缺损并重建左心室流出道,18＃带单瓣牛心包片补片重建肺动脉及右室流出道. 结果 术后无早期死亡,平均住院时间13.6d.发生低心排血量综合征1例,肝功能损害2例,二次开胸止血1例,均经相应的处理治愈.随访3例,随访时间3～5个月,复查超声心动图未发现左、右心室流出道梗阻;其中2例发现轻度至中度主动脉瓣关闭不全,但定期随访未发现继续加重. 结论 采用Nikaidoh手术治疗DORV和TGA患者,术后可获得良好的血流动力学效果,早期临床结果满意.     

两种不同手术方式矫治完全性大动脉错位伴有室缺和肺动脉狭窄的效果分析

目的 回顾性分析两种不同手术方式矫治完全性大动脉错位(TGA)伴有室间隔缺损(VSD)和肺动脉狭窄(PS)的效果.方法 对46例TGA/VSD/PS的患者进行手术矫治,其中采用主动脉根部移位/重建双室流出道术,即Nikaidoh术27例(N组),Rastelli手术19例(R组).N组采用自身心包补片重建右室流出道(RVOT),其中1例用同种异体带瓣管道Homograft;R组使用Homograft重建RVOT.两组均无手术前姑息手术史.结果 N组因术后严重心功能衰竭死亡1例(3.7%),R组无死亡.术后早期并发症的发生率两组相近.手术平均年龄N组(16.3±16)个月,R组(51±20)个月,N组明显小于R组(P=0.028).N组术后无明显残余左、右心室流出道梗阻(LVOTO、RVOTO),而R组有37%患者分别存在LVOTO或RVOTO(P＜0.05),但N组术后89%患者存在轻-中度肺动脉血反流现象,R组仅1例患者存在轻度反流(P＜0.05),两组手术早期心功能状况差异无统计学意义;随访期两组均无死亡,但R组有4例(23.6%)因LVOTO、RVOTO再手术治疗.结论 Nikaidoh术矫治TGA/VSD/PS患者,在解剖上更胜一筹,适宜于小的年龄患者.     

阜外医院71例Rastelli手术的近中期结果

目的总结分析阜外医院Rastelli手术的近中期结果。方法2010年5月至2017年3月阜外医院完成71例Rastelli手术治疗大动脉转位(TGA)或者右心室双出口(DORV)合并室间隔缺损(VSD)和肺动脉狭窄患儿,男48例,女23例;手术时年龄(4.7±2.7)岁,其中10例完全型大动脉转位;27例DORV,34例矫正型TGA(CTGA)。30例有手术史,其中Blalock-Taussig分流13例,双向Glenn手术17例。31例同时扩大室间隔缺损。外管道使用同种带瓣管道9例,牛颈静脉管道56例,自制Gore-Tex外管道6例;管道直径(17.9±3.3)mm。结果本组主动脉阻断(132.0±71.1)min,体外循环(209.0±83.4)min。机械通气时间(102.6±81.7)h。术后住院(13.6±12.8)天。早期死亡1例(1.4%)。早期并发症12例(16.9%),其中因Ⅲ度房室传导阻滞安装永久起搏器4例,心包积液开窗3例,延迟关胸3例,二次开胸2例。术后随访4个月~6.8年。1、5年生存率分别为97.2%,97.2%;1、5年免除右心室流出道狭窄(RVOTO)率分别为98.6%,84.1%;1、5年免除干预率分别为98.6%,90.0%。随访期间置换外管道1例,介入球囊扩张7例10次。所有患儿最后一次随访均未见左心室流出道狭窄,左心室到主动脉压差(10.5±8.8)mmHg。结论Rastelli手术适用于部分DORV、CTGA、TGA合并VSD和严重的肺动脉狭窄或肺动脉闭锁患儿,早期死亡比例低,中期效果好,但是远期常需要再手术,尤其是置换外管道。     

半旋转动脉干调转术治疗完全性大动脉转位合并室间隔缺损和肺动脉狭窄

目的 报道半旋转动脉干调转术治疗完全大动脉转位(TGA)合并室间隔缺损(VSD)和肺动脉狭窄(PS)的近期疗效.方法 2例病儿男、女各1例,分别为16岁和19个月.均为TGA/VSD/PS.采用半旋转动脉干调转术治疗.结果 2例病儿均生存.女病儿术后发生低心排出量综合征和毛细血管渗漏综合征,治疗后痊愈.男病儿术后恢复良好.术后分别随访10、9个月,心功能Ⅰ级.结论 半旋转动脉干调转术可有效治疗TGA合并VSD和PS.长期效果仍需观察.     

Lecompte术治疗小儿心室大动脉连接异常

目的 探讨和评价近年来Lecompte术在小儿心室大动脉连接异常的先天性心脏病（先心病）中的临床应用价值。方法 2000年1月至2006年4月18例心室大动脉连接异常的先心病病儿行Lecompte手术治疗。年龄3个月～9．2岁；体重4．5～27．0kg。大血管转位（D-TGA）、室间隔缺损（VSD）、肺动脉狭窄6例，右心室双出13（DORV）S例，永存动脉干7例。6例D-TGA中主动脉和肺动脉呈前后位4例，另2例与4例DORV主肺动脉呈右前左后关系，1例DORV主动脉动脉呈左前右后关系。7例因位于两半月瓣之间的漏斗（圆锥）隔妨碍建立VSD和主动脉之间隧道，遂切除漏斗隔。8例行经典Lecompte术。10例进行改良Leeompte术，前壁再用心包补片扩大。术毕病儿右心室和左心室收缩峰压比值（PRwPLV）为0．20～0．45。结果所有病儿均生存，5例表现为右心功能不全，术后彩色超声检查，2例右室流出道压差30．0—34．51mmg Hg／（1 mm Hg=0．133kPa）；2例行右肺动脉改良Leeompte术病儿压差37．5～47．3 mm Hg.分别随访3个月、2年，此2例病儿右肺动脉残余压差24．0—29．31／mm Hg。结论Lecompt手术可以减轻左心室流出道梗阻，避免使用人工管道，采用自体肺动脉重建肺动脉流出道具有生长潜能，特别适用于婴幼儿、甚至新生儿。     

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。     

改良主、肺动脉根部置换术治疗伴左室流出道梗阻的大动脉错位

目的 探讨一种新的主、肺动脉根部置换手术治疗合并室间隔缺损(VSD)、左室流出道梗阻(LVOTO)的大动脉错位(TGA).方法 4例伴LVOTO、VSD的TGA患者及1例伴LVOTO、VSD的右心室双流出口患者,被采用改良的Nikaidoh技术将主动脉和肺动脉根部完整互换移植、冠状动脉移植以及双心室流出道重建;其中2例房室异常连接患者被同期施行Senning手术.结果 所有患者手术均获成功,术后恢复良好.术后平均随访5.40个月,生长发育好.超声心动图检查提示心室功能良好,主动脉瓣无反流;2例患者肺动脉瓣有少量反流.结论 保留半月瓣的主、肺动脉根部置换术治疗伴LVOTO的复杂型TGA患者,不仅可获得解剖根治,同时解决了右心室流出道的非生长性问题,改进的冠状动脉再植技术扩大了Nikaidoh手术的适应范围,并获得很好的近期疗效.但其远期疗效仍需更大组的手术例数及更长期的随访来验证.     

The optimal procedure for the great arteries and left ventricular outflow tract obstruction. An anatomical study.

OBJECTIVE: To describe the optimal surgical strategy in heart specimens with transposition of the great arteries (TGA) and left ventricular outflow tract obstruction (LVOTO). METHODS: Thirty-three specimens with LVOTO were selected: TGA with intact ventricular septum (TGA/IVS) (10), TGA/VSD (21), and Taussig-Bing (2). RESULTS: LVOTO in TGA/IVS consisted of combinations of bicuspid pulmonary valve (four), subpulmonary fibrous ridge (four), obstructive muscular conus (two) and bulging muscular septum (four). Arterial switch operation (ASO) with LVOTO resection/valvotomy was feasible in nine hearts. Obstructive anterior papillary muscle prohibited LVOTO relief in one specimen. In TGA/VSD and Taussig-Bing LVOTO consisted of combinations of bicuspid (nine) or unicommissural (one) pulmonary valve, fibrous ridge (three), obstructive muscular conus (five), malaligned outlet septum (six), accessory mitral valve tissue (two), straddling mitral valve (two) and anterior mitral valve rotation (four). VSDs were subpulmonary in 13 (9 perimembranous, 4 muscular), subaortic in 3 (2 perimembranous, 1 anterior muscular), doubly committed in 2, inlet in 3 (2 perimembranous, 1 muscular), non-committed and anterior in 1, and finally 1 VSD extended both into inlet and subpulmonary outlet septum. LVOTO resection and ASO with VSD closure was possible in 10. In six specimens, both a Rastelli and a Nikaidoh operation were feasible. For two hearts, a Nikaidoh procedure was the only option, while Rastelli was considered optimal in another specimen. Mitral valve anomalies prevented LVOTO relief in four, only permitting for Senning/VSD closure (one) or univentricular palliation (three). CONCLUSIONS: LVOTO resection and pulmonary valvotomy frequently permits an ASO. Inlet VSD, impossibility of VSD enlargement, straddling mitral valve, distant aorta and small right ventricle make the Nikaidoh procedure the best option. Mitral anomalies preventing LVOTO relief can make biventricular repair impossible.     

Repair of transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction.

OBJECTIVES: This study was undertaken to compare the outcomes of the Lecompte procedure and Rastelli repair in the transposition of the great arteries (TGA) with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO) and to determine the risk factors associated with unfavorable events. METHODS: Over a 12-year period (April 1990-October 2002), 35 patients underwent complete repair for TGA, VSD, and LVOTO. Twenty-five patients (71%) underwent the Lecompte modification, and mean age and weight were 23.4+/-18.2 months and 10.2+/-3.0 kg. Ten patients (29%) underwent the Rastelli operation, and mean age and weight were 39.1+/-36.1 months and 13.8+/-6.8 kg. RESULTS: One early death (3%) occurred after the Lecompte procedure and no late death. The mean follow-up was 5.9+/-3.8 years. Eight patients in the Rastelli group (80%) underwent a late reoperation for obstruction of the extracardiac conduit, and in four of these patients, a reoperation for LVOTO was concomitantly required. Reoperation was also required in six patients of the Lecompte group (25%); five for right ventricular outflow tract obstruction (RVOTO) including one for LVOTO and two for VSD leakage, and one for mitral regurgitation and left pulmonary artery stenosis. The interval prior to reoperation ranged from 1.6 to 11.1 years, with a mean of 5.7+/-3.1 years. The actuarial figures for freedom from reoperation at 5 and 10 years were 40.0+/-15.5 and 26.7+/-15.0% after the Rastelli operation and 95.7+/-4.3 and 63.5+/-12.6% after the Lecompte procedure (P = 0.02). Multivariate analysis by Cox regression analysis revealed that the risk factors of RVOTO were a younger age at operation, the Rastelli operation, and ductus ligation during the operation. CONCLUSIONS: The Lecompte procedure and Rastelli repair provide satisfactory early and late results. However, substantial late morbidity is more associated with conduit obstruction, and LVOTO in Rastelli repair rather than Lecompte procedure.     

Surgery for transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction: European Congenital Heart Surgeons Association multicentre study

Objectives: Optimal surgical management for patients with transposition of the great arteries (TGA), ventricular septal defect (VSD) and left ventricular outflow obstruction (LVOTO) remains controversial. Although the Rastelli operation has been the most widely performed surgical procedure during the past decades, several studies have shown its suboptimal long-term prognosis. Other operations have been developed to improve results. This study was performed to compare the outcomes of the different surgical approaches for patients with TGA, VSD and LVOTO, as well as to determine risk factors for mortality and re-intervention. Methods: Records from 146 patients undergoing surgery from 1980 to 2008 from eight European hospitals were reviewed. Median age at operation was 21.5 months (range 0.2–165.1 months), and median weight was 10.0 kg (range 2.0–41.0 kg). Surgical procedures involved were the Rastelli procedure (82), arterial (24) and atrial (5) switch operation with relief of LVOTO, Réparation à l’Etage ventriculaire (REV) procedure (7) and the Metras modification (24), as well as the Nikaidoh procedure (4). Results: The overall survival was 88%, 88% and 58% at 1, 10 and 20 years, respectively. The overall event-free survival was 80%, 45% and 26% at 1, 10 and 20 years, respectively. The REV procedure and the Metras modification were found to have the best long-term results in both survival and event-free survival rates. Multivariate analysis revealed year of operation, non-commitment of the VSD and prolonged cardiopulmonary bypass (CPB) time as risk factors for mortality while age at surgery, year of operation and type of corrective surgery were risk factors for re-intervention. Conclusions: Different surgical approaches have been developed for patients with TGA, VSD and LVOTO. The REV procedure and the Metras modification were observed to have favourable long-term results in survival and event-free survival rates. Aortic translocation techniques such as the Nikaidoh procedure seem promising, but further studies will be needed to confirm this in the long term.     

Outcomes of 829 neonates with complete transposition of the great arteries 12-17 years after repair.

OBJECTIVE: Between 1985 and 1989, the surgical management of neonates with complete transposition (TGA) underwent a transition from atrial to arterial repair. We sought to examine the intermediate outcomes and their associated risk factors in neonates repaired during the era of transition. PATIENTS AND METHODS: Twenty-four institutions entered 829 neonates age less than 15 days in a prospective study. Diagnosis was simple TGA (n=631), TGA with ventricular septal defect (VSD) (n=167), TGA with VSD and pulmonary stenosis (TGA/VSD/PS) (n=30), or TGA with PS (n=1). Repair was by arterial switch (n=516), atrial repair (Senning=175, Mustard=110) or Rastelli (n=28). Time-related events were analysed by parametric hazard function modeling and incremental risk factors for mortality, re-intervention, and late functional assessment were sought. RESULTS: Survival estimates at 6 months, 5, 10, and 15 years are 85, 83, 83, and 81%, respectively. The hazard function for death after repair has two phases: an early rapidly declining phase and an ongoing constant one. Constant phase mortality is less likely after the arterial switch operation and in children with simple TGA. During follow up, at least one re-intervention was required in 167 children (pacemaker, n=35; percutaneous intervention, n=32; baffle re-intervention, n=27; re-operation, n=125). Freedom from re-intervention at 6 months, 5, 10 and 15 years is 93, 82, 77, and 76%, respectively. Of survivors, 87% have been followed up to the last 3 years, including an assessment of functional ability of 562 children (83%). Functional class 15 years after repair is class I in 76%, II in 22%, III in 2%. The proportion in functional class I decreased over time. Psychosocial deficits, especially learning disorders are prevalent. CONCLUSIONS: Survival 15 years after TGA repair is good with most children functioning well, and results are best after an arterial switch operation. There is an ongoing risk of death that is less after the arterial switch operation. With the exception of Rastelli patients, the likelihood of survivors needing re-intervention after 5 years is low. There is need for improved neurodevelopmental outcomes.     

Surgical problems in d-TGA with VSD and PS associated with insertion of tricuspid valve chordae to the infundibular septum--the Rastelli operation by translocation of the infundibular septum

Twelve (37.5%) out of 32 patients who underwent surgical repair of complete transposition of the great arteries (d-TGA) with ventricular septal defect (VSD) and pulmonary stenosis (PS) from 1978 to 1987 had insertion of tricuspid valve (TV) chordae to the infundibular septum, i.e. superior margin of the VSD, which precluded the ordinary Rastelli type operation. Of these 12 patients early 5 patients (ages: 2-11 mean 7 years) underwent atrial switch operations with VSD closure and pulmonary valvotomy with or without fibromyotomy, and recent 6 patients (age: 4-8, mean 6 years) underwent the Rastelli operation (3 cases) or REV (3) by translocating the infundibular septum with TV chordae to the right ventricular side of the internal conduit. None died in hospital in the former group and 1 in the latter. Pulmonary/systemic ventricular peak pressure ration (Ppv/sv) was 0.75 +/- 0.22 (mean +/- standard deviation) in the atrial switch group v. 0.49 +/- 0.08 in the Rastelli/REV group 1 month postoperatively. The higher Ppv/rv in the atrial switch group was attributed to the pressure gradient across the pulmonary outflow tract, which was 37.8 +/- 16.6 mmHg in the atrial switch v. 16.7 +/- 4.2 mmHg in the Rastelli/REV (p less than 0.05). No pressure gradient was demonstrated between left ventricle (LV) and aorta in the both groups. Pulmonary wedge pressure was higher in the atrial switch group (13.8 +/- 1.9 mmHg) than in the Rastelli/REV (10.0 +/- 2.7 mmHg) (p less than 0.05). Systemic atrioventricular (AV) valve regurgitation was noted in 2 of the atrial switch group but none in the Rastelli/REV.(ABSTRACT TRUNCATED AT 250 WORDS)     

完全性大动脉转位的个体化外科治疗

目的 研究不同类型完伞性大动脉转位(TGA)的个体化外科治疗策略及其效果.方法 1998年3月至2009年10月,共收治各类TGA患者127例(158例次),男性97例,女性30例.年龄生后4 h～17岁,平均(25±37)个月,其中<3个月56例66例次;体质量2.7～47.5 kg,平均(8±8)kg.初期手术行Glenn手术14例(其中3例行双侧Glenn手术),主-肺动脉分流术14例,肺动脉环缩术8例,房间隔缺损扩大+肺动脉环缩+主-肺动脉分流术15例.终期手术包括Seaning手术5例,一期Switch手术32例,二期Switch手术11例,Switch手术+室间隔缺损修补术20例,Switch手术+室间隔缺损镶嵌手术1例,Nikaidoh手术3例,Rastelli手术13例,Fontan手术18例,心内隧道等其他手术4例.结果 20例患者接受初期手术后现正在等待或已放弃二期手术,21例患者接受2期手术,5例接受3次及以上手术.66例次患者术后延迟关胸.采用腹膜透析9例,呼吸机使用时间2 h～16 d.全组早期死亡12例(病死率9.4%),死因包括术后低心排出最5例,肺动脉高压危象2例,术中出血2例,冠状动脉畸形1例,感染合并自发性肝破裂1例,肾功能衰竭1例.109例患者术后随访1个月～12年,6例失访,远期2例死亡.随访患者中,10例有不同程度的并发症,3例已再次手术,随访效果好.余存活病例,心功能正常,生长发育良好.结论 根据TGA患者解剖条件,采用个体化治疗策略,制定不同的手术方案,可以明显提高患者的手术成功率和远期生存率.     

Rastelli operation as one stage anatomical correction for infants with complete transposition of the grat arteries and ventricular septal defect

We recently adopted the Rastelli operation as a one stage anatomical correction for three infants with transposition of the great arteries and associated with ventricular septal defect (TGA+VSD). In all, ventricular septal defect was enlarged by excision of the conus septum to avoid obstruction of intraventricular tunnel and Hancock valved conduits of 18 mm in diameter were used for reconstruction of pulmonary trunk. The postoperative function of the left ventricle proved to be better than that of the right ventricle, as the systemic ventricle, after the Mustard procedure for TGA+VSD. Our results show that the Rastelli operation is effective as a one stage anatomical correction in infants with TGA+VSD.     

Rastelli operation as one stage anatomical correction for infants with complete transposition of the great arteries and ventricular septal defect

We recently adopted the Rastelli operation as a one stage anatomical correction for three infants with transposition of the great arteries and associated with ventricular septal defect (TGA + VSD). In all, ventricular septal defect was enlarged by excision of the conus septum to avoid obstruction of intraventricular tunnel and Hancock valved conduits of 18 mm in diameter were used for reconstruction of pulmonary trunk. The postoperative function of the left ventricule proved to be better than that of the right ventricle, as the systemic ventricle, after the Mustard procedure for TGA + VSD. Our results show that the Rastelli operation is effective as a one stage anatomical correction in infants with TGA + VSD.