小儿ccTGA解剖纠治术中应用双向Glenn/半Mustard心房调转双调转手术的初步研究CSCD

目的 评估先天性矫正型大动脉转位中应用半Mustard心房调转手术及双向Glenn手术完成双调转手术的风险及收益.方法 为了避免完全Senning手术的并发症并且辅助右心血流动力学,采用一种改良的心房调转手术.2014年1月到2016年3月,7例患儿完成此种改良双调转手术的解剖矫治.5例合并肺动脉闭锁患儿施行了Rastelli-心房调转手术,2例施行了动脉-心房双调转手术.心房调转使用半Mustard手术占同期解剖矫治术中的26.9％（7/26）.结果 全组无近期死亡.随访7～21个月,无死亡,无心房板障相关的再次手术或窦房结功能不全.术后三尖瓣反流轻至中度6例,中度1例.右心室-肺动脉管道无明显梗阻.结论 改良心房调转手术完成双调转手术初期结果良好.半Mustard和双向Glenn手术的减少板障及窦房结相关的并发症,手术方法相对简单,能改善右心功能和预后.     

Double Switch术治疗先天性矫正性大动脉转位二例

目的 介绍解剖矫治先天性矫正性大动脉转位的手术特点。方法 2例矫正性大动脉转位患者,1例9个月,合并室间隔缺损、肺动脉高压,行Senning加动脉调转术;1例23岁,合并室间隔缺损、肺动脉狭窄、三尖瓣关闭不全,行Rastelli加Senning术。结果 2例患者全部成活,其中1例术前出现Ⅲ度房室传导阻滞,安装永久起搏器,术后超声检查心室功能正常,心内隧道和心外管道通畅。无三尖瓣反流。结论 Double Switch手术是一种理想的治疗先天性矫正性大动脉转位的方法。     

矫正型大动脉转位合并心内畸形的手术方法和效果

目的 探讨双调转手术和传统修复术治疗矫正型大动脉转位合并心内畸形的效果.方法 2002年4月至2006年12月,19例矫正型大动脉转位合并心内畸形患者接受手术治疗,男性14例,女性5例;年龄2～22岁,平均8.6岁.合并的心内畸形包括:室间隔缺损18例,右心室双出口1例,肺动脉狭窄17例,房间隔缺损4例,肺动脉高压2例.手术方式包括双调转手术解剖矫正修复15例,传统修复术治疗心内畸形4例.结果 双调转术后死亡1例,发生严重低心排血量综合征2例,一过性房室传导阻滞1例,反复胸腔积液2例.传统心内修复术后无死亡病例,发生严重低心排血量综合征1例.双调转手术存活者随访6个月～4年,均为窦性心律,心功能NYHA分级Ⅰ～Ⅱ级,射血分数51%～68%.传统心内修复术存活者随访1年,3例心功能达Ⅰ～Ⅱ级,射血分数52%～61%;1例心功能Ⅲ级,射血分数40%.结论 双调转手术解剖修复矫正型大动脉转位合并心内畸形早中期效果良好,严格选择手术适应证和手术时机是提高手术效果的关键.对于右心室功能较好,无三尖瓣畸形的患者,如果存在影响双调转效果的因素,仍应选择传统心内修复手术.     

心房及大动脉双调转术治疗先天性矫正型大动脉转位

目的 总结先天性矫正型大动脉转位心房及大动脉双调转术治疗的初步经验.方法 先天性矫正型大动脉转位行心房及大动脉双调转手术17例.8例行Senning+大动脉调转术,年龄11个月～11岁,中位年龄3.7岁,其中7例合并肺动脉高压,1例合并肺动脉瓣下狭窄,3例曾行肺动脉环缩术-左心室锻炼.9例行Senning+ Rastelli手术,年龄5～24 岁,中位年龄9.0岁,其中5例行室缺扩大术.结果 Senning+大动脉调转手术组主动脉阻断183～282 min,术后呼吸机辅助14～984 h,引流208～1496 ml.术后完全性房室传导阻滞1例,安装永久性心脏起搏器后病愈出院;右侧膈肌麻痹1例,行膈肌折叠术后病愈出院.Senning+ Rastelli手术组主动脉阻断132～380 min,术后呼吸机辅助18～276 h,引流108～1780 ml,术后ECMO辅助循环1例,因低心排综合征死亡1例.结论 对于年龄较大的先天性矫正型大动脉转位病儿,心房及大动脉双调转术近期治疗效果满意.     

双调转术(Double-Switch手术)的临床应用

目的 探讨Double-Switch手术方法的临床应用。方法 采用Double-Switch手术方法纠治房室连接和心室大动脉连接不一致的复杂先天性心脏病病儿3例，其中1例为纠正型大血管错位、室间隔缺损、肺动脉狭窄伴轻度三尖瓣反流，2例为房室连接不一致、右心室双出口、肺动脉狭窄、室间隔缺损、三尖瓣轻一中度反流。心房内采用Senning术，心室内采用Rastelli术的方法，从解剖上彻底予以纠治。结果 手术均取得成功，恢复顺利，分别术后11d、18d和21d出院。术后随访心电图窦性节律，超声检查示心功能正常，腔静脉、肺静脉血流通畅，同种带瓣管道和心内隧道血流无梗阻。结论 对房室连接和心室大动脉连接不一致病例，特别是解剖右心室功能不全和三尖瓣反流者，应首选Double-Switch手术。     

大动脉调转术治疗肺动脉瓣下室间隔缺损型右心室双出口的初步体会

右心室双出口合并肺动脉瓣下室间隔缺损（TaussigBing畸形，TBA）为复杂先天性心脏病，手术治疗较难，尤其在婴幼儿期。早年多数采用心内隧道纠治的方法，远期疗效差。近年来，随着手术操作技术的提高，采用大动脉调转术（ASO）治疗的成功率明显提高，远期效果佳。现将我们开展这类手术的初步体会总结如下。     

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.     

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。     

心室双出口手术治疗临床研究

目的：探讨心室双出口的手术治疗效果。方法：回顾性分析72例心室双出口病人手术治疗方法及治疗效果。右室双出口71例中SDD型64例，ILL型3例，SDL型3例，IDD型1例；左室双出口1例（ILD型）。采用左心室-主动脉心内隧道连接61例，全腔静脉-肺动脉连接2例，左心室-主动脉心内隧道并右心室-肺动脉心外管道连接3例，Glenn分流6例。结果：死亡2例。1例术后3个月发现残余室间隔缺损，予再次手术缝合。余者效果良好，无远期死亡及相关并发症发生。结论：根据心室双出口的不同类型，选择最佳手术方式及掌握手术时机是成功的重要因素，重建通畅良好的左或右室流出道是提高手术成功率的关键因素。     

改良Rastelli手术治疗右心室双出口合并左前位主动脉

目的 探讨右心室双出口合并左前位主动脉患者采用改良Rastelli的手术效果。方法 对10例右心室双出口患者采用改良Rastelli手术,9例为右心室双出口合并右前位主动脉,1位为镜面右位心型右心室双出口。除1例外,9例患者均有肺动脉瓣及瓣下狭窄。室间隙缺损位于肺动脉下或远离两大动脉。体外循环下行改良Rastelli手术,经右心室切口用涤纶片或人工血管片修补空间隔缺损,形态左心室至主动脉的内隧道。切断主肺动脉,闭合近端。用带瓣同种肺动脉或主动脉及人造血管,建立右心室流入道三关瓣环下方至主肺动脉的外管道。结果 10例患者术后恢复良好,超声心去科显示所有内隧道及外管道功能正常,无血流梗阻征象。结论 改良Rastelli手术是治疗右心室双出口合并左前位主动脉的满意术式,它可以彻底矫治肺动脉瓣及瓣下狭窄和右向左分流。     

Staged Approach for Congenitally Corrected Transposition of the Great Arteries Associated with Dextrocardia and Nonconfluent Pulmonary Artery

Abstract   We completed double switch operation (Senning plus Rastelli procedure; SLL) after staged palliative operations for SLL, dextrocardia, congenitally corrected transposition of the great arteries ventricular septal defect, atrial septal defect, and nonconfluent pulmonary artery. The patient had undergone three preparatory operations including central pulmonary artery reconstruction with autologous pericardium and morphologic right ventricle-pulmonary artery shunt and then double switch operation was performed. During the double switch operation, the previous conduit which was established as right ventricle-pulmonary artery shunt in the third palliation was replaced by a larger conduit. This conduit was used as Rastelli external conduit to avoid coronary artery injury. When systemic venous chamber was reconstructed, suture line pouch technique was used, in which dog ears were made at the sites of superior and inferior vena cava orifices, respectively, to prevent systemic venous return obstruction. Pulmonary venous chamber was reconstructed without any augmentation, which reduced the possibility of postoperative arrhythmia. Systemic and pulmonary venous pathway obstructions were not observed after the operation. Postoperative arrhythmia was not detected.     

Surgical correction of atrioventricular discordance--results and follow-up]

Between January 1977 and October 1991, 47 consecutive patients with atrioventricular discordance, ventriculoarterial discordance, and two ventricles underwent intracardiac repair at the Heart Institute of Japan. Their ages at operation ranged from 2 months to 21 years (average 9.2 years) and period of follow-up ranged from 1 month to 168 months (average 65.1 months). There were five basic anatomic types according to the associated anomalies, grouped into (1) situs solitus, ventriculoarterial (V-A) discordance or double-outlet right ventricle (DORV), ventricular septal defect (VSD) and pulmonary outflow tract obstruction (POTO) (31 patients); (2) situs solitus, V-A discordance and VSD (6 patients); (3) situs solitus, V-A discordance and tricuspid regurgitation (TR) (2 patients); (4) situs inversus, V-A discordance or DORV, VSA and POTO (7 patients); (5) situs inversus, V-A discordance and VSD (1 patient). TR was evident preoperatively in 13 patients (28% of 47 patients), one of whom had Ebstein's anomaly. The conventional operation, which consisted of closure of VSD (34 patients), posterolateral pulmonary outflow enlargement (2 patients), tricuspid valvuloplasty (2 patients), and tricuspid replacement (1 patient) using the anatomical right ventricle as the systemic ventricle, was performed in 35 patients (RV group). The anatomic correction (so-called "double switch operation"), the new alternative operation using the anatomical left ventricle as the systemic ventricle, was achieved in 12 patients (LV group). Anatomic correction consisted of Senning and RV-PA ECR in 3, Mustard and RV-PA ECR in 4, Senning and arterial switch in 2, and Mustard and arterial switch in 1 patient.(ABSTRACT TRUNCATED AT 250 WORDS)     

Combined Rastelli and atrial switch procedure: a 10-year follow-up.

We performed a combined atrial (Mustard) and ventricular (Rastelli) repair on a previously palliated patient with situs inversus, atrioventricular discordance, ventricular septal defect, and pulmonary atresia. The suitability and durability of this operative strategy is supported by the satisfactory hemodynamic and functional status of the patient 10 years later.     

A CLINICAL COMPARISON OF ARTERIAL AND ATRIAL REPAIRS FOR TRANSPOSITION OF THE GREAT ARTERIES: EARLY AND MIDTERM SURVIVAL AND FUNCTIONAL RESULTS

Background : The early experience in correction of simple transposition of the great arteries (TGA) involved redirection of atrial inflow to re-establish systemic and pulmonary blood flow, the basis of the Senning and Mustard operations. Since 1984, however, the arterial switch operation (Jatene) has been increasingly used. It appears that with experience the peri-operative mortality rate for arterial repair is comparable to that for atrial repairs, while the late morbidity rates have generally favoured arterial repair, with sequelae such as obstruction of venous inflow, dysrhythmias, systemic atrioventricular valve dysfunction and impaired systemic ventricle function being more common following atrial repairs. Methods : A retrospective review of patients surgically treated for TGA at the Prince Charles Hospital. Results : Between April 1973 and July 1994, 103 patients with simple TGA underwent surgical correction using one of three standard approaches currently advocated (arterial switch/Jatene, Senning or Mustard operations). Fifty-one Jatene, 31 Senning and 21 Mustard operations were performed. Associated procedures included closure of ventricular septal defect, right and left ventricular outflow tract resection and pulmonary artery band removal. The coronary artery anatomy was found to be suitable in all except two patients when the Jatene operation was planned; both these patients had a Senning procedure without mortality. There were nine perioperative deaths (4 Jatene, 2 Senning, 3 Mustard), and five late deaths (1 Jatene, 1 Senning, 3 Mustard). Follow-up was maintained to a mean time of 60.5 months (range, 9 days to 203 months). In the Jatene group there were five anastomotic stenoses (all involving the neo-pulmonary artery), three cases of arryhthmias (two postoperative supraventricular tachycardia and one nodal rhythm) and two pulmonary valve stenoses. In the Senning group there were four anastomotic stenoses (three involving pulmonary vein and one causing superior vena cava (SVC) inlet obstruction) and four cases of arryhthmias (with one requiring permanent pacemaker insertion). In the Mustard group there were two cases of SVC obstruction and two permanent pacemaker insertions for bradycardia. Conclusions : These early and midterm results suggest that the arterial switch operation has comparable overall mortality to, and less morbidity than, the atrial repairs for TGA with the retention of left ventricle to systemic arterial connection, which may provide improved long-term results.     

Results of the double switch operation in the current era

BACKGROUND: In patients with atrioventricular and arterioventricular discordance congenitally corrected transposition, the morphologically right ventricle may progressively deteriorate while functioning in the systemic circuit. The double switch operation has been proposed to limit this functional deterioration. METHODS: From October 1993 to August 1998, the records of 27 patients with congenitally corrected transposition were reviewed. Age at operation ranged from 3 months to 55 years. Associated defects included ventricular septal defects in 18, pulmonary atresia in 7, and pulmonary stenosis in 11 patients. Twenty-two patients had double switch operations (10 arterial switch plus Senning procedures and 12 Rastelli plus Senning procedures). Five patients were not candidates for the double switch. Before the double switch, 6 patients required pulmonary artery banding and 10 had functioning systemic to pulmonary artery or cavopulmonary shunts. RESULTS: There was no early or late mortality. Two patients required pacemaker implantation, both later regained normal sinus rhythm. Tricuspid valve function improved in all patients except one. Moderate left ventricular dysfunction developed 5 months postoperatively in 1 patient. CONCLUSIONS: The double switch operation can be performed in selected patients with minimal early morbidity and mortality. Longer follow-up is necessary to determine whether this complex approach is indeed warranted.     

Surgical management of double-outlet right ventricle

From 1977 to 1983, 32 consecutive patients, ranging in age from 15 days to 24 years, underwent operations for double-outlet right ventricle. Twenty patients had a palliative operation either to increase (12 cases) or to reduce (eight cases) pulmonary blood flow: Ten of them have subsequently undergone total repair, and in another six correction was delayed because of possible incremental operative risk factors, such as multiple ventricular septal defects or the need for an extracardiac conduit. Four patients with multiple, complex associated intracardiac anomalies are currently considered to have uncorrectable defects. A total of 22 patients underwent correction either primarily (12) or after palliation (10). Intraventricular tunneling was performed in 16 patients with a subaortic ventricular septal defect and in one with a doubly committed ventricular septal defect. Seven of these had pulmonary stenosis and five had reconstruction of the right ventricular outflow by means of a patch (three) or a conduit (two); among this group, five also had enlargement of the ventricular septal defect. In three patients with a subpulmonary defect and in one with a remote ventricular septal defect, all of them without pulmonary stenosis, total repair was achieved by a Senning, a Mustard, or an arterial switch operation. Finally, the only patient with atrioventricular discordance and pulmonary stenosis had insertion of a left ventricle-pulmonary artery conduit. No operative deaths were observed after palliation, but one patient died of intrapulmonary hemorrhage after total repair (4.5%). Major postoperative complications included detachment of the ventricular septal defect patch in one patient and late progression of pulmonary vascular obstructive disease in another. No late deaths have been recorded. Surgical repair of double-outlet right ventricle can be accomplished with gratifying early and late results, the risk of operative death being below 5%. The outcome in patients with subaortic ventricular septal defect appears particularly favorable, despite the extensive intracardiac procedures required for total correction. An early intervention is recommended to prevent development of pulmonary vascular obstructive disease and to avoid massive cardiac hypertrophy and fibrosis, which may cause late rhythm disturbances and impede the intracardiac repair.     

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.