Double—Switch术治疗矫正型大动脉转位

目的：使矫正型大动脉转位（CTGA）不仅从生理上,而且从解剖上得到了纠正。方法：资料为美国波士顿儿童医院心脏外科收治的9例CTGA患者,年龄2－16个月,体重3.1-11kg。术前均行彩色多普勒超声心动图和心导管造影检查,心排血量和心功能测定,9例患者均施行Double-Switch术,合并的其它畸形都予以纠治。结果：9例患者均存活,术后彩色多普勒超声心动图示左、右心功能明显改善。结论：小婴儿CTGA应尽可能早期选做Double-Switch术,使心功能尽可能适合机体的循环机制。     

双调转术治疗先天性矫正型大动脉转位疗效分析

2002年4月至2003年4月，我们应用双调转手术治疗4例先天性矫正型大动脉转位(cCTGA)病儿，收到满意的治疗效果，现报道如下。     

左室训练术治疗先天性矫正型大动脉转位的临床疗效分析

目的评价左室训练术治疗先天性矫正型大动脉转位(cCTGA)的临床疗效。方法 2005年5月至2011年5月,阜外心血管病医院对24例左心室退化的cCTGA患者行左室训练术,其中男13例,女11例;年龄0.17～22.00(3.73±4.35)岁;体重5.10～61.00(15.71±10.95)kg。主要合并畸形:三尖瓣关闭不全(TR)23例(轻度11例、中度7例、重度5例),限制型室间隔缺损18例,房间隔缺损5例,卵圆孔未闭5例,动脉导管未闭4例,肺动脉瓣轻度狭窄5例,主动脉弓缩窄1例。术前经超声心动图、心血管造影或心导管检查确诊,形态学左心室舒张期末内径(mLVEDD)8～32(21.56±6.60)mm,形态学左心室(mLV)后壁厚度2～7(4.29±1.52)mm,形态学左心室与形态学右心室(mRV)压力比(PmLV/mRV)0.12～0.65(0.41±0.12)。手术均采用胸骨上端小切口或胸骨正中切口,在全身麻醉下完成肺动脉环缩术。结果术毕测压PmLV/mRV 0.57～0.93(0.76±0.10);全组患者无住院死亡,出院时超声心动图检查提示:双心室结构和功能良好,室间隔位置较术前略向mRV侧移位,mLVEDD较术前略增大,TR较术前减轻。术后随访1～35个月,无远期死亡。所有患者一般情况好,生命体征平稳,心功能分级(NYHA)Ⅰ～Ⅱ级,mLVEDD 14～40(26.17±7.11)mm,mLV后壁厚度4～9(4.95±1.44)mm,PmLV/mRV 0.52～0.98(0.72±0.16),TR较术前明显减轻,其中14例患者完成二期心房大动脉双调转术。结论左室训练术应用于左心室退化的cCTGA患者安全有效,mLV压力负荷及后壁厚度增加,mLV腔扩大,且TR显著减轻。     

先天性矫正型大动脉转位的外科治疗进展

先天性矫正型大动脉转位(congenitally corrected transposition of the great arteries，ccTGA)指房室(atrioventricular，AV)和心室大动脉(ventriculoarterial，VA)连接的不一致,ccTGA是一种少见的先天性心脏畸形，约占先天性心脏病的1％。大多数ccTGA病儿合并其他心脏畸形。ccTGA临床表现和自然病程的变化取决于其合并的畸形。     

矫正性大动脉转位纠治术的麻醉处理

我院于 1987年 4月～ 1999年 6月在气管内插管静脉复合全麻下为 71例矫正性大动脉转位病人实行纠治术 ,术后死亡 9例 ,无麻醉死亡。现将麻醉处理报告如下。临床资料一般资料　本组男 4 1例 ,女 30例 ,年龄 3～ 37岁 ,体重14～ 74 5ｋｇ。属功能矫正型大动脉左转位 (ＳＬＬ)型 5 3例 ,功能矫正型大动脉右转位 (ＩＤＤ) 16例 ,解剖矫正大动脉右转位 (ＳＬＤ)型、解剖矫正大动脉左转位 (ＩＤＬ)型各 1例。合并房缺 (ＡＳＤ) 2 1例 ,室缺 (ＶＳＤ) 6 4例 ,肺动脉狭窄 (ＰＡＳ) 6 7例 ,动脉导管未闭 (ＰＤＡ) 2例 ,左房室瓣关闭不全 2例。术前发…     

Rastelli手术治疗大动脉转位伴室间隔缺损

目的介绍Rastelli手术治疗大动脉错位伴室间隔缺损的经验.方法全组49例中男29例,女20例.平均年龄5.6岁;平均体重17.2kg.完全性大动脉转位31例,纠正性大动脉转位18例;伴肺动脉狭窄45例,伴肺动脉高压4例.均在低温体外循环下行Rastelli手术.二期根治5例.体外循环灌注(178.5±52.5)min;主动脉阻断(109.2±38.3) min.结果手术早期死亡6例,死亡率12.2%.死因为肺动脉高压危象、肾衰、心律紊乱和严重低心输出量综合征.术后并发症有心律紊乱、肺动脉高压危象、蛛网膜下腔出血、脑功能紊乱、肾衰及多脏器功能衰竭、心包或胸腔积液、感染等.CICU平均监护7.3 d.随访中因同种带瓣大动脉(VHC)感染死亡1例.结论完全性大动脉转位手术中,右室流出道直切口有利于心内隧道修补室间隔缺损;纠正性大动脉转位手术中,解剖右室径路显露缺损较好且易避开传导系统,但不利于术后心功能恢复.而解剖左室径路修补室间隔缺损的房室传导阻滞发生率高;大于4岁者手术宜选择大号VHC可减少再次手术几率;对无长段左室流出道狭窄的完全性大动脉转位病婴,可在动脉换位术基础上行肺动脉瓣叶交界切开或Konno术以解除左室流出道梗阻.为防止术后功能性二尖瓣反流,对伴肺动脉狭窄的纠正性大动脉转位病儿,提倡心房-大动脉双换位手术.     

矫正型大动脉转位伴心脏畸形外科治疗

自１９８５年９月至１９９４年３月手术治疗３３例矫正型大动脉转位伴心脏畸形病人，其中男２１例，女１２例，年龄１～３５岁（平均１１岁）。其中ＳＬＬ型３１例，ＩＤＤ型２例，手术修复室间隔缺损２８例，肺动脉狭窄２９例（１４例应用外带瓣管道，其中９例同种主动脉带瓣管道，５例为带戊二醛处理的猪肺动脉瓣的人工管道），修补房间隔缺损１５例，左侧房室瓣关闭不全成形２例，动脉导管未闭缝合和改良Ｆｏｎｔａｎ手术各１例。     

老年先天性矫正型大动脉转位行心瓣膜置换术一例

临床资料患者,女,72岁。体检发现心脏杂音20余年,因活动后胸闷、气促半个月余人院就诊。患者既往身体健康,无高血压病、糖尿病等。入院体检：心率90次／分,呼吸18次／分,血压113／66mmHg;神智清楚,自主体位,口唇无紫绀,颈静脉无怒张,颈动脉搏动不明显;     

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]     

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。     

Double switch operation for congenitally corrected transposition of the great arteries after two-staged pulmonary artery banding

We describe a case of congenitally corrected transposition of the great arteries (cc-TGA) successfully performed by the double switch operation after two-staged pulmonary artery banding (PAB). An eleven-year old boy diagnosed with cc-TGA underwent the first PAB at that age, followed by the second PAB one year later. Because of severe ventricular dysfunction and arrhythmia of the anatomic left ventricle, the intension of one-stage PAB was abandoned. Cardiac catheterization data from after the adequate second PAB provided the surgical indication for the anatomical correction and double switch operation (Senning+Jatene procedure) and this was successfully performed at age 14. Although cardioversion was required to treat supraventricular tachycardia in the early period after surgery, the patient was discharged from hospital and remains in good clinical condition at the last follow-up at 5 years with normal sinus rhythm and good biventricular function.     

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.     

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.     

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.     

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.     

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.