Results of the double switch operation for congenitally corrected transposition of the great arteries.

OBJECTIVE: To determine the outcomes for a program that utilizes the double switch operation as the primary approach for congenitally corrected transposition. METHODS: The records of 46 consecutive patients from a single institution who had undergone a double switch operation from October 1993 to March 2002 were reviewed. The records of 24 patients who were evaluated during the same period and felt not to be candidates for the double switch operation or who are awaiting double switch after pulmonary artery banding were also reviewed. RESULTS: The median age at operation was 28 months (range 2 months to 16.3 years). Associated defects included ventricular septal defect 40, pulmonic stenosis 13 and pulmonary atresia 16. Twenty-six patients underwent an arterial switch operation combined with a Senning procedure while 20 patients underwent combined Rastelli and Senning procedures. Before the double switch, 12 patients had required pulmonary artery banding and 21 patients had systemic to pulmonary artery shunts. The median duration of stay in the intensive care unit was 3.5 days (range 2-60 days) and the median duration of total hospital stay was 8 days (range 5-60 days). There were no hospital deaths; one patient died 5 months after discharge due to an arrhythmogenic cardiac arrest during a median follow-up of 24 months [long-term survival 98% (95% CI 89-100%)]. CONCLUSIONS: The double switch operation may be performed with excellent hospital and long-term survival. The theoretical advantages of this procedure which enables the morphologic left ventricle and mitral valve to support a systemic pressure load must be established by careful follow-up of these patients.     

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.     

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。     

Surgical treatment of transposition of the great arteries: the arterial switch operation

Transposition of the great arteries (TGA) is one of the most common congenital cardiac anomalies resulting in cyanosis. Various atrial-level physiological procedures were developed in the late 1950s and early 1960s, including the Senning and Mustard procedure. In 1975, Jatene et al. reported the first successful arterial switch procedure in a patient with d-TGA and ventricular septal defect. In 1977, Yacoub et al. introduced a two-stage repair comprising initial pulmonary artery banding to retain the left ventricle, supplemented by a systemic-pulmonary artery shunt, thereby potentially expanding the arterial switch procedure to a much wider population of patients. In 1983, the concept of the primary neonatal arterial switch procedure was introduced by Castaneda et al. Since then, the primary artery switch procedure in neonates and early infants has become the standard for the treatment of TGA. This is due to the fact that the only the arterial switch procedure allows complete anatomical repair. In Japan, 102 neonates with simple TGA underwent the arterial switch procedure, with hospital death occurring in 21 (20.6% mortality rate) according to the 1998 annual report. In Okayama University Hospital, 48 neonates, underwent the arterial switch procedure, with 1 hospital death (2.1% mortality rate) and no late deaths since 1991.     

Late reoperations after neonatal arterial switch operation for transposition of the great arteries.

The arterial switch operation has become the treatment of choice for neonates with transposition of the great arteries. Currently, the early mortality rate is low as well as the need for early reoperation because of surgical failures; in our experience with 803 neonates, these risks were 3.8% and 1.5%, respectively. The late outcome in terms of survival and functional status is excellent. However, surgical repair is far from anatomical and potential late defects were identified as soon as this procedure was introduced: obstruction of the neo-pulmonary outflow tract, development of obstructions of the reimplanted coronary arteries, dysfunction of the neo-aortic valve, and progressive left ventricular dysfunction. Actually, late reoperations are required in 5-10% of patients (4.5% in our experience with a mean follow-up of 5.8 years). The more frequent indications for reoperation are coronary lesions and right ventricular outflow tract obstructions. Coronary obstructions are, in most cases, detected in patients without any clinical or echocardiographic evidence of myocardial ischaemia. Coronary lesions are progressive and repeated coronary evaluation at regular intervals is necessary. Reoperation is indicated when myocardial ischaemia, at rest or under stress, is demonstrated at myocardial imaging. Satisfactory results can be achieved by surgical coronary patch angioplasty; in selected cases, mammary bypass may be necessary. Right ventricular outflow tract obstruction is related either to inadequate growth of the pulmonary anastomotic site, or to inadequate growth of the whole new right ventricular outflow tract in patients with associated aortic arch obstruction. Reoperation is indicated when significant obstruction (gradient >50 mmHg) is detected at routine echo-Doppler evaluation. Although neo-aortic root dilation and minimal aortic valve insufficiency are common, reoperation for severe neo-aortic valve dysfunction is, to date, very rarely necessary. Whether this will remain the case in the decades to come requires further evaluation. Left ventricular function is maintained in the vast majority of patients. Reoperation may be indicated in some patients for other reasons: mitral valve malformation, tracheo-bronchial compression or pulmonary hypertension.     

Combined arterial switch and Senning operation for congenitally corrected transposition of the great arteries: patient selection and intermediate results

OBJECTIVE: Late results after traditional methods of repair of congenitally corrected transposition of the great arteries are poor. The combined arterial switch and Senning (double switch) operation may improve outcomes by using the morphologically left ventricle and mitral valve in the systemic circulation. In this report we review patient selection and intermediate results after the double switch operation for congenitally corrected transposition of the great arteries. METHODS: Since 1993, a total of 35 patients with congenitally corrected transposition of the great arteries with two ventricles of adequate size and no valvular pulmonary stenosis were potential candidates for a double switch operation. Eleven were not yet in need of further treatment, and 1 died during evaluation. The remaining 23 patients were entered into a protocol leading to anatomic repair. Their hospital records were reviewed, and follow-up data were obtained to evaluate early and intermediate outcomes. RESULTS: The 23 patients were candidates for anatomic repair because of right ventricular dysfunction or tricuspid regurgitation (n = 15) or associated uncorrected defects (n = 8). Pulmonary artery banding was performed in a total of 15 patients, either for left ventricular retraining (n = 11) or for congestive heart failure (n = 4). In 2 patients, aged 12 and 14 years, retraining was unsuccessful because of left ventricular dysfunction. Four patients with banding are currently awaiting repair. Eight patients proceeded to undergo double switch operations without preliminary pulmonary artery banding. To date, 17 patients have undergone double switch operations, with no early or late mortality. One patient required cardiac transplantation for progressive left ventricular failure after a preliminary banding and double switch operation done at 7 years of age. Ventricular function and tricuspid regurgitation remained stable or improved in all other cases. No patient has surgically acquired arrhythmias or significant residual hemodynamic conditions. All patients are alive and clinically well at a mean follow-up of 36 months (range 1 month-8 years). CONCLUSIONS: Congenitally corrected transposition of the great arteries with a normal pulmonary valve and two adequate ventricles can be managed with combined arterial switch and Senning operation with excellent intermediate results. Reconditioning the left ventricle may not be suitable for older patients. Late follow-up will be necessary to determine whether this management strategy provides a survival advantage for these patients.     

Modified arterial switch operation by spiral reconstruction of the great arteries in transposition

Background. Spiral relationship of the normally related great arteries (SRGA) has never been reconstructed in an arterial switch operation.

Methods. From March 1998 to April 1999, 9 consecutive cases of transposition of the great arteries (TGA) family (from 2 days to 1.6 years old) underwent arterial switch operations with SRGA at our hospital. Two had a congenitally corrected TGA (plus atrial redirection). Lecompte maneuver was not used in all. The posterior wall of pulmonary trunk was not divided but three were reattached, two of whom had had previous pulmonary trunk banding. Thus the wall was shared between the great arteries facing each other.

Results. All survived the operation. Supraaortic stenosis was balloon-dilated in 2 cases of early series, but technical modifications later were able to avoid it. Angiogram showed smooth flow into SRGA without upward and anterior tilting of the pulmonary bifurcation. All great and coronary arteries were patent. All were doing well on follow-up (16.5 ± 4.2 months).

Conclusions. We concluded that the techniques to relocate the coronary arteries using common wall and in situ switch could also be applied to pulmonary arterial reconstruction, so that SRGA can be resumed in TGA.     

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.     

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。     

Successful neonatal double switch in symptomatic patients with congenitally corrected transposition of the great arteries

Anatomic repair is the standard surgical approach to congenitally corrected transposition of the great arteries. However, timing to perform the procedure remains controversial. We present 2 cases of congenitally corrected transposition of the great arteries and Ebstein's-like anomaly of the tricuspid valve presenting with heart failure. Both cases had successful anatomic repair during the neonatal period.     

Late outcome after arterial switch operation for complete transposition of great arteries with left ventricular outflow tract obstruction

BACKGROUND: Long-term follow-up of patients who underwent arterial switch operation for complete transposition of great arteries with anatomic left ventricular outflow tract obstruction (LVOTO) has rarely been brought into the focus. METHODS: Of 299 patients who underwent an arterial switch operation between January 1991 and January 2001, 23 patients had anatomic LVOTO. Age ranged from 4 days to 18 years (median 90 days) and weight ranged from 2.6 to 35 kg (median 4.3 kg). Surgical management included arterial switch operation, closure of ventricular septal defect wherever indicated, and excision of LVOTO. RESULTS: Among patients with preoperative LVOTO there were 2 early deaths and 8 patients had mild neoaortic regurgitation at the time of discharge. Follow-up ranged from 8 months to 9 years (mean 60 +/- 12 months). In 4 patients who had mild neoaortic regurgitation at discharge, the regurgitation progressed to moderate or severe degree after a follow-up of 22 to 72 months. In 1 patient mild mitral regurgitation present at the time of discharge progressed to severe mitral regurgitation. This patient subsequently underwent double valve replacement. CONCLUSIONS: Presence of preoperative anatomical LVOTO in patients undergoing arterial switch operation predicts high incidence of postoperative neoaortic regurgitation.     

Surgical results of the Senning operation and arterial switch operation for complete transposition of the great arteries

From March 1980 to August 1997, 42 patients with complete transposition of the great arteries (TGA) type I and II underwent arterial switch procedure (n = 20) or Senning (n = 22) procedure in our institution. In arterial switch procedure group, there were two operative death. No late death occurred, and one patient underwent reoperation and one underwent balloon pulmonary angioplasty for right sided obstruction. The freedom from the intervention rate for right sided obstruction was 83.0% at 5 years, subsequently. In Senning procedure group, operative death occurred in two patients with TGA type II, and one patient with TGA type II and two patients with type I died because of cardiac events in the late stage. In this group, two patients died from accident. The survival rate excluding accidental deaths was 77.7% and 77.7% at 5 and 10 years, respectively. However, those patients who died in the late stage had severe pre-operative and post-operative complication. In conclusion, mid-term results of arterial switch procedure is satisfactory, however, reintervention rate was not so low. Long term results of Senning procedure without preoperative or postoperative complication was satisfactory.     

双调转术纠治小儿纠正型大血管错位伴肺动脉狭窄

Objective To compare the results of surgical repair for congenitally corrected transposition of the great arter-ies(ccTGA) with pulmonary stenosis(PS), and to analyze the risk factors that may affect early results and surgical technique. Methods From Aug. 2001 to Dec. 2008, 21 patients with ccTGA and PS were treated in our hospital. They aged 3.5 months to 6.3 years [(31 ± 18) months], weighted 6.28 kg [(13.1± 6.5) kg]. Fifteen cases had paramembranous ventricular sep-tal defect (VSD), 1 noncommitted VSD and 5 subpulmonary VSD. The repairs comprised of Senning and Rastelli operation. RV-PA reconstruction was done by xenopericardial conduit in 13 patients. RV-PA direct anastomosis plus pericardial patch in 6 patients and homograft patch with autopulmonary valve in 2 patients. Results All the patients were alive. During 2 - 5 years follow-up, blood flow rate in superior vena cava increased to 1.8 - 2.2 m/s in 3 cases. One of them needed reoperation to re-lease the stenosis and the rest 2 were in follow-up. Tow patients had right ventricular outlet obstruction with a pressure gradient of 30 -45 mmHg were in follow-up. There was no other stenosis and valve insufficiency. Conclusion Double-Switch opera-tion is practical and impressive in treating of CCTGA with PS. It is important to evaluate the size of VSD and the reconstruction of RV-PA. But it still need more cases and longer time to evaluate the long-term effects.     

双调转术纠治小儿纠正型大血管错位伴肺动脉狭窄

目的 分析和评估纠正型大血管错位伴肺动脉狭窄病儿采用双调转(Double-Switch)手术的治疗效果.方法 2001年8月至2008年12月采用Double-Switch手术行纠正型大血管错位伴肺动脉狭窄21例.其中男11例,女10例;年龄3.5个月至6.3岁,平均(31±18)个月;体重6～28 kg,平均(13.1±6.5)kg.室间隔缺损(VSD)为膜周型15例,远离大动脉1例,肺动脉下5例.轻度二尖瓣反流2例,中度反流2例.采用Senning+Rastelli手术方法 .13例右心室与肺总动脉的连接采用牛颈静脉管道.结果 全组均生存.体外循环转流156～287 min,平均(202.0±35.8)min;主动脉阻断93～161min,平均(138.0±19.8)min.均无完全性房室传导阻滞.随防2～5年,上腔静脉流速增快3例(1.8～2.2)m/s,其中1例术后2.6年再次手术解除上腔静脉梗阻,余2例在随防中;肺静脉回流均无明显梗阻;牛颈静脉管道通畅,解剖右室流出道连接处梗阻2例,压力阶差在30～45 mm Hg(1 mm Hg=0.133kPa),尚在随防中.结论 Double-Switch手术纠治纠正型大血管错位伴肺动脉狭窄的复杂型先心病,取得了较好效果,但仍需进一步随访,以观察这类复杂手术方法 的长期疗效.     

双调转术纠治小儿纠正型大血管错位伴肺动脉狭窄

Objective To compare the results of surgical repair for congenitally corrected transposition of the great arter-ies(ccTGA) with pulmonary stenosis(PS), and to analyze the risk factors that may affect early results and surgical technique. Methods From Aug. 2001 to Dec. 2008, 21 patients with ccTGA and PS were treated in our hospital. They aged 3.5 months to 6.3 years [(31 ± 18) months], weighted 6.28 kg [(13.1± 6.5) kg]. Fifteen cases had paramembranous ventricular sep-tal defect (VSD), 1 noncommitted VSD and 5 subpulmonary VSD. The repairs comprised of Senning and Rastelli operation. RV-PA reconstruction was done by xenopericardial conduit in 13 patients. RV-PA direct anastomosis plus pericardial patch in 6 patients and homograft patch with autopulmonary valve in 2 patients. Results All the patients were alive. During 2 - 5 years follow-up, blood flow rate in superior vena cava increased to 1.8 - 2.2 m/s in 3 cases. One of them needed reoperation to re-lease the stenosis and the rest 2 were in follow-up. Tow patients had right ventricular outlet obstruction with a pressure gradient of 30 -45 mmHg were in follow-up. There was no other stenosis and valve insufficiency. Conclusion Double-Switch opera-tion is practical and impressive in treating of CCTGA with PS. It is important to evaluate the size of VSD and the reconstruction of RV-PA. But it still need more cases and longer time to evaluate the long-term effects.     

Results of arterial switch operation for transposition of great arteries with regard to coronary pattern

One of the factors determing the postoperative results of arterial switch operation (ASO) for complete transposition of great arteries (TGA) is coronary event (CE). We analyzed the risk factors of CE after ASO in patients whose coronary patterns excluding Shaher type 1. The subjects were 44 patients operated from 1994 to 2007. As for the coronary reconstructive technique, procedures by Mee or Aubert were conducted on patients with Shaher 5A intramural type. The conventional technique was conducted on other patients, with or without trapdoor technique. In all patients, the pulmonary artery was reconstructed mainly with autopericardium using Lecompt maneuver. The mean observation period was 4.0 +/- 3.1 years, and the number of patients showing CE was 14 (32%) at 1.6 years after surgery on the average. The risk factors of CE and the operative procedures were discussed.     

Prevalence and predictors of neoaortic regurgitation after arterial switch operation for transposition of the great arteries

BACKGROUND: The fate of the native pulmonary valve after arterial switch operation is still unknown and may become a cause for a secondary aortic valve operation during adult life. We evaluated the prevalence and predictive factors associated with neoaortic valvular regurgitation by a retrospective study of children who underwent arterial switch operation for transposition of the great arteries. METHODS: The onset of neoaortic valvular regurgitation was correlated with demographic data, cardiac anatomy, surgical technique, and postoperative ventricular function. The size of the neoaortic root and ascending aorta was measured in a selected subset of patients. RESULTS: Among 253 survivors, 173 were eligible for the study. After a median follow-up time of 8.2 years, 61 patients showed echocardiographic or angiographic evidence of valvular incompetence, which was progressive in 14 cases; this led to surgical intervention in 2 patients, and there was 1 operative death. At multivariate analysis, the onset of valvular regurgitation was correlated with the trap-door technique for coronary reimplantation (P <.01). A smooth transition from the aortic sinus to the ascending aorta, with loss of the normal sinotubular junction geometry, may be associated with valvular incompetence. CONCLUSIONS: After arterial switch operation, there is an increasing frequency of neoaortic regurgitation, which may lead to significant valvular dysfunction later in life. The trap-door type of coronary reimplantation is associated with an increased risk for valvular dysfunction, possibly because of a distortion of the sinotubular junction geometry. For this reason, we recommend the punch technique for repair in all but the most complicated coronary pattern.