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。     

Double switch with reversed patrick-mcgoon for corrected transposition of the great arteries with double outlet right ventricle

A 4-year-old boy diagnosed with a double outlet right ventricle with transposition of the great arteries in a discordant atrioventricular connection. The anatomical characteristics of the ventriculoarterial relationships resulting from leftward deviation were quite similar to a mirror image of those in the malformation reported by Patrick and McGoon. Hence, it was most appropriate to perform the double switch operation associated with Patrick-McGoon’s intraventricular rerouting and Senning’s atrial switch. This intraventricular rerouting could be performed without damaging the conduction system because of existence of the anterior node. The cardiac catheterization 1 month after the operation showed no pressure gradient of the new reconstructed left ventricular outflow tract, good left ventricular function and no significant pressure gradient of the right ventricular outflow tract. The patient has been staying in class 1 of New York Heart Association for more than 3 years after the operation.     

双调转术治疗矫正型大动脉转位合并心内畸形

目的总结双调转手术治疗矫正型大动脉转位（cTGA）合并心内畸形的经验。方法2002年4月至2004年6月，行双调转手术治疗cTGA合并心内畸形病儿7例，其中男6例，女1例；年龄4—15岁，平均8．4岁。SLL型6例，IDD型1例。合并室间隔缺损6例，右室双出口1例，继发孔房间隔缺损2例，肺动脉狭窄6例；3例SLL型为右旋心，1例IDD型为左旋心。手术方式包括改良Senning＋Rastelli 4例，Mustard＋Rastelli＋双向Glenn手术、Senning＋Rastelli手术、改良Senning＋Switch手术各1例。结果术后早期死亡1例，为cTGA合并室间隔缺损、肺动脉高压，行改良Senning＋Switch手术者，死因为左心功能衰竭。早期主要并发症有低心排血量综合征、房室分离各1例，胸腔积液和低蛋白血症2例。随访2—24个月，恢复良好，复查多普勒超声心动图、心电图和X线胸片显示，窦性心律5例，结性心律1例，心功能均为Ⅰ级。结论双调转手术可以达到解剖矫治cTGA合并心内畸形，早期手术死亡率低，中、远期心功能效果良好。SLL型病例采用改良Senning心房内调转手术效果优于Senning和Mustard手术。     

Early prophylactic pulmonary artery banding in isolated congenitally corrected transposition of the great arteries

Objective: Mid-term evaluation of an aggressive surgical management of isolated congenitally corrected transposition of the great arteries (ccTGA) by pulmonary artery (PA) banding in early infancy. Methods: Between 2001 and 2009, 11 asymptomatic patients (seven neonates and four infants) underwent a dilatable, partially adjustable, homemade PA banding for ccTGA with intact ventricular septum. PA band circumference was correlated to body weight (22 mm + 1 mm kg⁻¹) and ideally adjusted to obtain flat septal geometry. Mean age at operation was 1.5 ± 1.4 months. Results: There was no hospital mortality. Mean ventilation time and intensive care unit (ICU) stay were 20 ± 9 h and 2.6 ± 1.5 days, respectively. Five patients required postoperative inotropic support. One late death occurred suddenly at 4 months; normal biventricular function and no tricuspid regurgitation were noted at last follow-up, 1 week before death. Mean follow-up was 21.5 ± 26 months. Mean band velocity increased over time from 2.65 ± 0.7 m s⁻¹ postoperatively to 3.7 ± 0.3 at 6 months and 4.5 ± 0.4 m s⁻¹ at 2 years. Tricuspid regurgitation remained stable in seven patients, decreased in three and worsened in one. Flat septal geometry was obtained in all patients after the third postoperative month. One patient underwent a double-switch procedure at 7 years due to suprasystemic morphologically left ventricular pressure. The postoperative course was uneventful. Conclusions: In neonates with isolated ccTGA, prophylactic PA banding is safe and carries a low morbidity. At mid-term evaluation, tricuspid valve function is stabilised or improved and systemic competence of the left ventricle is maintained, thus allowing double switch if indicated.     

Bilateral pulmonary artery banding for transposition of the great arteries complex with coarctation

Surgical approaches for transposition of the great arteries with aortic arch obstruction include primary repair and two-stage repair. However, neither approach provides a satisfactory outcome. We report a case of patient who underwent two-stage repair, wherein arterial switch operation combined with aortic arch reconstruction was preceded by bilateral pulmonary artery banding; this yielded good outcomes. This approach safely avoids primary repair in the neonatal period and allows for the opportunity to evaluate right ventricle outlet tract stenosis before the definitive repair.     

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.     

快速二期动脉转位术纠治新生儿完全型大动脉转位

目的总结快速二期动脉转位术的临床应用效果.方法 2002年9月至2003年5月,5例完全型大动脉转位患者行快速二期动脉转位术.手术平均年龄83.0±72.2 天,平均体重4.7±0.9 kg.由于求诊时已经超过最佳手术年龄,左心室退化,不能承受体循环压力,则先行肺动脉环缩和体肺动脉分流术,术后6～9天行第二期动脉转位术.结果一期手术中,1例术后32小时发生室上性心动过速,少尿,经腹膜透析和药物处理无效死亡;其余4例分别在术后6～9天行二期动脉转位术,无死亡.术后随访2～10个月,生长发育良好,超声心动图检查显示心内无残余分流,主动脉和肺动脉干吻合口通畅,左心室射血分数(EF)0.68～0.77,短轴缩短率(FS)0.24～0.37,1例主动脉瓣轻度反流.结论对失去最佳手术时间的新生儿完全型大动脉转位,行快速二期动脉转位术是最佳的选择.     

Modification of the arterial switch operation for transposition of the great arteries with complex coronary artery patterns

The arterial switch operation has evolved into the treatment of choice for most forms of transposition of the great arteries (TGA). Recently reported operative mortality of the procedure has fallen to the range of 1.1%–6.0%, even for complex forms of TGA. Despite advancement in the technical aspects of the procedure, certain anatomical variations of the coronary arteries, such as a single coronary orifice and/or intramural coronary artery, are still considered surgical risks in many centers. Optimizing the surgical technique for relocating these challenging variations of the coronary anatomy is key to improving the surgical outcomes for the procedure. In this review, the surgical modifications of the arterial switch operation for TGA associated with complex patterns of the coronary arteries are examined. This review was submitted at the invitation of the editorial committee.     

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.     

Results of a new technique of arterial switch operation for transposition of great arteries with single coronary artery

Background Transposition of great arteries (TGA) with Single Coronary artery is one of the high risk groups for Arterial Switch Operation (ASO). To eliminate this risk, we have innovated a new technique of ASO with insitu coronary re-allocation. We present our experience with this new technique. Methods From September 1998 to October 2005, ten consecutive cases of TGA with single coronary artery were operated employing this new technique. Their age ranged from 8 days to 9 months; their weight ranged from 2.6 to 5.8 Kgs. ASO was done by transecting the great arteries just above the commissures. For coronary re-allocation, hockey stick shaped incisions were made in the facing sinuses of the proximal aorta and the pulmonary artery. These flaps were sutured in such a way that the coronary ostium was committed to the neo-aorta. Results There was no in-hospital mortality. The follow-up ranged from 4 months to 7 years. All patients had follow-up echocardiogram at regular intervals, which showed no significant right or left ventricular outflow obstruction, no regional wall motion abnormalities and no, neo-aortic or neo-pulmonary regurgitation. Conclusion This new coronary re-allocation technique avoids problems related to coronary translocation such as traction and kinking. It spares the need for dissection of proximal coronary artery and its branches, and thereby eliminates the risk of development of fibrosis and stenosis. The same technique can be used regardless of the sinus of origin of the coronary artery. It is a reliable and reproducible technique. The early results were excellent.     

Functional outcome of anatomic correction of corrected transposition of the great arteries

Objective: Anatomic correction of corrected transposition of the great arteries, utilizing the morphologic left ventricle as a systemic pumping chamber, is considered the preferred method. The purpose of the study was to analyze the intermediate functional outcome following anatomical correction. Methods: Between 1997 and 6/2010, 23 patients with corrected transposition of the great arteries and associated lesions underwent anatomical correction. Seventeen (74%) and six patients (26%) had situs solitus {S,L,L} and situs invs {I,D,D}, respectively. Fifteen patients (65%) had undergone 18 palliations before the corrective operation. The median age at palliation was 0.23 years, with a range of 0.016–8.4 years. A corrective, modified Senning–arterial switch procedure was performed in nine patients, 13 patients underwent a modified Senning–Rastelli procedure, and in one patient a combination of modified Senning and aortic translocation (Bex/Nikaidoh) was used. The median age at the corrective operation was 2 years (from 0.3 to 15.7 years). Results: There was no mortality or heart transplant within the mean follow-up of 3.4 years. Freedom from reintervention was 77% at 5 years. There were no signs of obstruction of the systemic and pulmonary venous tunnels. The function of both ventricles was normal in all patients, even in the four patients who required retraining of the left ventricle. Mild aortic regurgitation was noticed in three patients. Preoperatively detected significant tricuspid regurgitation either disappeared or became trivial after the operation in all the six patients. All patients except two are in sinus rhythm; one patient is pacemaker-dependent preoperatively and one is pacemaker-dependent postoperatively. There were no clinically apparent neurological problems. All patients, but one, are in the New York Heart Association (NYHA) class I. Conclusions: Anatomic correction of corrected transposition of the great arteries can be performed in selected patients without mortality and with acceptable morbidity. The mid-term functional outcome is excellent, resulting in normal ventricular function, even in retrained left ventricles, and minimal incidence of complete heart block. The long-term function of the aortic valve, intraventricular tunnels, conduits, and ventricles requires close surveillance.     

Modification of the Senning procedure in the double-switch operation: The triangular double-door technique

We developed a modified Senning procedure in the double-switch operation for the patients with congenitally corrected transposition of the great arteries (ccTGA). In our technique, the right atrial (RA) free wall is not used as a baffle for draining systemic venous blood to the left atrium. Instead, a patch material is used for the baffling. A wide communication between the pulmonary venous chamber and RA is created by making the triangular double door with the RA-free wall, and the window is closed with in situ pericardial flap. We have successfully adopted this technique in our recent two consecutive ccTGA patients.     

完全性大动脉转位一期动脉调转术后ICU延迟恢复的临床特征及影响因素分析

目的 总结完全性大动脉转位(TGA)一期大动脉调转(ASO)术后早期临床特点及围术期处理策略,评价年龄结构及畸形复杂程度对术后恢复进程的影响,并分析导致术后ICU延迟恢复的相关风险因素.方法 回顾性分析2015-2017年我院连续231例行一期ASO手术的TGA患儿临床资料,其中男165例、女66例,年龄3d至10岁....     

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.     

快速与长期双期大动脉调转术在室间隔完整大动脉转位中应用的对比研究

目的通过比较快速和长期双期大动脉调转术(ASO)的组间特点,总结发生左室退化后室间隔完整大动脉转位(TGA-IVS)的手术治疗经验。方法纳入2007年1月至2019年1月主要诊断为TGA-IVS并行双期ASO的患儿41例,其中男25例、女16例,平均年龄17.9个月(7 d至7岁)。根据双期手术间隔时间,分为快速双期ASO组(19例)和长期双期ASO组(22例)。比较两组患儿临床效果。结果两组患儿的年龄、体重、ASO术前血氧饱和度、训练术前左室舒张期末内径、ASO术前左室后壁厚度差异均有统计学意义(P<0.05)。患儿年龄>1岁是进行长期双期ASO的独立危险因素。结论长期双期ASO对年龄>1岁和左心室退化严重的患儿具有良好的疗效。     

Aortic translocation, Senning procedure and right ventricular outflow tract augmentation for congenitally corrected transposition, ventricular septal defect and pulmonary stenosis

The management of congenitally corrected transposition of the great arteries and associated lesions is frequently challenging. Significant pulmonary stenosis is a contraindication to the conventional double-switch. Instead repair may be accomplished by the Rastelli–Senning procedure, using an extracardiac conduit to achieve continuity between the morphological left ventricle and the pulmonary arteries. This however can be accompanied by conduit and intra-ventricular baffle-related problems that can necessitate surgical re-intervention and lead to late mortality. We describe the use of aortic translocation, Senning procedure and reconstruction of the right ventricular outflow tract using autologous tissue and valved homograft to facilitate anatomical correction in congenitally corrected transposition. The advantages of this technique in this group of patients and the implications for conduction tissue are discussed.