Damus-Stansel-Kaye procedure: technical modifications.

The Damus-Stansel-Kaye procedure has been applied for the relief of outflow tract obstruction caused by a restrictive bulboventricular foramen or subaortic stenosis in patients with complex univentricular heart disease. The procedure may also be part of a biventricular repair of a Taussig-Bing transposition of the great arteries. This report details technical modifications of the procedure to ensure unobstructed blood flow from the pulmonary artery to the aorta and to maintain the integrity of the pulmonary and aortic valves.     

Additional aortopulmonary anastomosis for subaortic obstruction in the Rastelli-type repair for the Taussig-Bing malformation

In the Taussig-Bing malformation, residual subaortic obstruction from a hypertrophied subaortic conus is often a problem at the time of intraventricular repair. To alleviate this problem, during a Rastelli-type repair involving an extracardiac valved conduit and an intraventricular connection from the ventricular septal defect to both aortic and pulmonary outflows, an additional side-to-side anastomosis between the ascending aorta and the proximal portion of the divided pulmonary artery can be performed, creating a supplementary outflow for the left ventricle. This method was used in 2 patients who had anteroposterior transposition of the great arteries, with 1 long-term survivor.     

Growth of the hypoplastic ascending aorta after radical palliation

Surgical treatment of hypoplasia of the ascending aorta is a difficult problem. An approach using proximal pulmonary artery to distal aorta bypass with distal pulmonary artery banding was employed in 19 patients with an ascending aorta less than 6 mm in diameter but a patent aortic outflow. There were 11 male and 8 female neonates and infants, and weight ranged from 2 to 4.5 kg (median weight, 3.5 kg). Four patients had transposition of the great arteries, 12 had subaortic stenosis, and 5 had interrupted aortic arch. There was one operative death. Cerebral cyanosis developed in 3 of the 4 patients with transposition of the great arteries and necessitated additional operations within the first year postoperatively. Therefore, palliation with a pulmonary artery to descending aorta conduit and banding was abandoned in such patients. All 14 surviving patients with normally related great arteries had successful palliation and growth of left heart structures. Subsequent procedures included a Fontan/Damus procedure in 1 patient, patch aortoplasty and pulmonary arterioplasty in 5 patients, arch reconstruction with aortic valvulotomy and pulmonary arterioplasty in 2, double arch reconstruction in 2, and simple patch aortoplasty with plans for subsequent pulmonary arterioplasty and ventricular septal defect closure in 1. Results demonstrate that in patients with a hypoplastic ascending aorta, this radical method of palliation can result in growth of right and left heart structures and thus provides the possibility of biventricular repair.     

The Damus-Stansel-Kaye procedure. Should the aortic valve or subaortic valve region be closed?

Two patients (one with transposition of the great arteries and another with Taussig-Bing anomaly) underwent the Damus-Stansel-Kaye procedure (Group I). Significant aortic valve insufficiency developed postoperatively in both patients. In contrast, seven patients with a univentricular heart and subaortic stenosis from a variety of reasons underwent creation of an aortopulmonary window (Group II), a procedure very similar to the proximal main pulmonary artery-aortic root anastomosis of the Damus-Stansel-Kaye procedure. Aortic valve insufficiency had not developed after up to 7 years of follow-up in this group (average 43 months). Postoperative angiograms suggest that aortic valve incompetence in Group I may have been caused by prolapse of the aortic valve. The valvular structures are subjected to high systolic pressures and face a dilated, low-pressure right ventricle. Aortic root distortion may have contributed, as well. In Group II patients, the aortic valve structures face a small, thick-walled chamber. The orientation of the aortic valve vis-a-vis the right ventricle changed postoperatively in Group I but not in Group II patients. Our experience suggests that the aortic valve or subaortic valve region should be closed at the initial repair in patients with low pulmonary vascular resistance who are undergoing the Damus-Stansel-Kaye procedure, to minimize the need for reoperation for aortic valve insufficiency.     

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。     

大动脉调转术后吻合口梗阻的影响因素

目的 分析大动脉调转术后主动脉和肺动脉吻合口梗阻的影响因素.方法 1999年12月至2007年12月,行大动脉调转术(ASO)331例,术后生存288例.228例平均随访(20.4±18.6)个月,随访率79.2%.根据ASO术后超声报告所测主、肺动脉吻合口流速的大小,对完全性大血管错位室间隔完整型(TGA/IVS),完全性大血管错位伴窒间隔缺损(TGA/VSD),右室双出口伴肺动脉瓣下室间隔缺损、肺动脉高压(Taussig-Bing)和快速二期大动脉调转术(Stage-Switch)的随访资料分别分析其主、肺动脉吻合口的梗阻情况.对ASO手术后的各类疾病的主动脉和肺动脉吻合口流速,按流速<2 m/s,2～3 m/s,>3 m/s的病例百分数进行统计分析.结果 4种疾病分类的随访结果示主动脉吻合口流速差异有统计学意义(P=0.034),肺动脉吻合口流速差异无统计学意义(P>0.05).肺动脉吻合口流速增快发生率比主动脉吻合口高.Taussig-Bing组发生率高,Stage-Switch组发生率低.手术时病婴年龄越小(≤12 d),主动脉、肺动脉吻合口流速越易增快.随访时间延长,流速增快的发生率会逐渐提高.全组6例由于左、右流出道梗阻而再次手术.结论 ASO术后,肺动脉吻合口梗阻发生率较高.ASO术后必须定期随访,注意观测吻合口的生长情况.     

Modified Rastelli procedure for double outlet right ventricle with left-malposition of the great arteries: report of 9 cases

BACKGROUND: Double outlet right ventricle with L-malposition of the great arteries is a rare type of double outlet right ventricle. This article reviews our experience in treating this disease surgically. METHODS: Between September 1995 and May 1999, 9 patients with double outlet right ventricle [S, D, L] underwent modified Rastelli procedure at Fu Wai Hospital. In all patients, the two great arteries originated completely from the right ventricle. The malformation was associated with subaortic conus and L-transposition of the great arteries. The ventricular septal defect (VSD) was subpulmonary in 3 patients, and non-committed in 6. All but one patient had either pulmonary valvular stenosis or subpulmonary stenosis. No patient had any type of palliative operation before. A right ventriculotomy was made to repair the VSD with a Dacron or vascular prosthesitic patch, and an intraventricular tunnel was made between the left ventricle and the aorta. The main pulmonary artery was divided and the proximal end was closed. A homograft conduit was implanted between the inlet of the right ventricle and the main pulmonary artery. In the Rastelli procedure, the conduit is usually positioned between the right ventricular outflow tract and the pulmonary artery. RESULTS: All patients survived and recovered uneventfully. Echocardiography demonstrated that all intraventricular tunnels and valved conduits were functioning well. The results were satisfactory. CONCLUSIONS: Modified Rastelli procedure is an optimal method for surgically treating double outlet right ventricle with left-malposition of the great arteries. It can completely correct the right ventricular outflow tract stenosis, and right to left shunt, and avoid injuring the right coronary artery.     

A case of tracheo-bronchial stenosis after extended end-to-end aortic arch anastomosis for interrupted aortic arch treated with suspension of the ascending artery and pulmonary artery

A 9-day-old boy had pulmonary artery banding and extended end-to-end aortic arch anastomosis for ventricular septal defect (VSD) and type A interrupted aortic arch. Severe dyspnea gradually developed. At 3 months of age, intracardiac repair of VSD was performed. Weaning from the ventilator was difficult. Endoscopic examination and chest CT revealed stenosis of the right and left main bronchi and compression of tracheal bifurcation and the right and left main bronchi by the ascending aorta and pulmonary artery. Suspension of the ascending aorta and pulmonary artery was performed 15 days after VSD closure. Nine days after this procedure, the patient was weaned from respirator. Postoperative course was uneventful. Bronchial stenosis may be caused from extended end-to-end aortic arch anastomosis.     

Primary repair of interrupted aortic arch and severe aortic stenosis in neonates

Two infants, aged 36 days old (Case 1) and 18 days old (Case 2) with interrupted aortic arch types B and A, respectively, and with severe aortic stenosis, were successfully operated on by use of pulsatile cardiopulmonary bypass. The great arteries were normally related in Case 1 and were transposed in Case 2. Repair involved the following procedure: ligation of the patent ductus arteriosus, restoration of aortic continuity with an 8 mm polytetrafluoroethylene graft, placement of an internal patch to tunnel all left ventricular blood from the left ventricle through the ventricular septal defect into the pulmonary artery in Case 1 and patch closure of the ventricular septal defect in Case 2, transection of the main pulmonary artery, anastomosis between the proximal pulmonary artery and the ascending aorta, and interposition of a valved conduit between the right ventricle and the distal pulmonary artery. The operative field could be approached easily through a median sternotomy. Postoperative cardiac catheterization revealed satisfactory anatomical and hemodynamic results in both cases.     

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。     

What is necessary for improvement of arterial switch operation?

We report long-term results of the arterial switch operation (ASO) and postoperative complications related to operative procedure. Between 1998 and 2007, ASO were performed in 42 patients [transposition of the great arteries (TGA) with intact ventricular septum:21, TGA with ventricular septal defect (VSD):13, Taussig-Bing anomary (TBA):7, and double outlet right ventricle with noncommitted VSD:1]. Hospital death occurred in 1 patient (2.4% mortality rate) due to low cardiac output syndrome (LOS) caused by prolonged aortic clamp, who had TGA with VSD, coactation of aortic arch and right ventricular outflow obstruction. Four patients required re-operation (freedom from re-operation rate was 84.7%). Two had pulmonary stenosis and 1 needed right ventricular outflow tract (RVOT) reconstruction with transannular patch. His pulmonary valve used for previous VSD closure was diminished. The other required muscle resection of RVOT. Five patients presented grade II or more neo-aortic insufficiency and 2 of them were TBA. Both these TBA, rerouting of left ventricular outflow tract (LVOT) to the aorta was challenging, they showed LVOT obstruction postoperatively. One underwent aortic valve plasty and the other had residual VSD closure. None had coronary event or abnormality in coronary arteriography. Selection of surgical procedure should be based on morphologic features in order to improve surgical outcome.     

Anatomic repair of anomalies of ventriculoarterial connection associated with ventricular septal defect. II. Clinical results in 50 patients with pulmonary outflow tract obstruction

From November 1980 to November 1985, 50 patients underwent anatomic repair of anomalies of ventriculoarterial connection associated with ventricular septal defect and pulmonary outflow tract obstruction. The technique used was one that we have previously described, which we call REV. The principles of this technique are resection of the infundibular septum, construction of a tunnel connecting the left ventricle to the aorta, and direct anastomosis, without a prosthetic conduit, of the pulmonary arterial trunk with the right ventricle. The tunnel is situated beneath the aortic valve and occupies very little space in the right ventricular cavity. Age at operation ranged from 4 months to 13 years (mean 3.5 years). Twenty-six patients had a classic type of transposition of the great arteries; all other patients had various types of anomalies of ventriculoarterial connection in which it was impossible, after the intraventricular connection of the left ventricle to the aorta, to use the natural pulmonary orifice for the pulmonary outflow tract reconstruction. There were nine hospital deaths (18%) and one late death. Twenty-six of 29 patients whose follow-up time exceeded 1 year had an excellent clinical result. No stenosis of the aortic outflow tract was found. Four patients had significant pressure gradients on the pulmonary outflow tract. Our present experience with REV suggests that this technique allows anatomic repair in a wide variety of anomalies of ventriculoarterial connection associated with ventricular septal defect and pulmonary outflow tract obstruction, even in infants, with an acceptable rate of mortality and morbidity.     

A valved conduit replacement for a calcified homograft 12 years following a Rastelli operation: a case report

A 25 year-old male with transposition of great arteries, ventricular septal defect and pulmonary stenosis successfully underwent Rastelli procedure with aortic homograft in 1969. After the procedure he grew up and became an engineer without any complaints. However, gradually his homograft degenerated with calcification and the pressure gradient between pulmonary artery and right ventricle increased to 77 mmHg in systolic phase. Twelve years after the conduit repair, we operated upon and enlarged the ventricular septal defect, repaired the residual interventricular shunt, removed his homograft and, replaced it with the Hancock valved conduit from the right ventricular outflow tract through cardiopulmonary bypass. On postoperative cardiac catheterization, the pressure gradient decreased, and the patient returned to his work.     

Arterial switch in simple and complex transposition of the great arteries

Arterial switch for repair of transposition of the great arteries was performed on 53 patients since October 1983. These patients were divided into three groups: group I, 25 infants with an intact ventricular septum who had primary repair in the first month of life (2 to 34 days of age, mean 9.7 +/- 6.6); group II, 13 patients with an intact ventricular septum who had anatomic repair after a preliminary procedure (pulmonary artery banding in 13, shunt in 10, atrial septectomy in 1); and group III, 15 infants with transposition of the great arteries and ventricular septal defect. In group III, six patients had Taussig-Bing abnormality, nine had previous pulmonary artery banding, three had coarctation of the aorta repaired earlier in life, and four were less than 2 weeks old. Overall early mortality was 9.4% (5/53: group I 8%, group II 7.6%, group III 13.3%). Two late deaths occurred in group II 10 and 12 weeks postoperatively after infection and high fever. A third late death 18 weeks postoperatively was due to aspiration in an infant with Goldenhar's syndrome. Mortality and morbidity decreased significantly after an initial learning period (no deaths from July 1985 to March 1987 overall, and none in the last 15 infants operated on in group I). The surviving 45 patients are doing well. All have normal sinus rhythm. Two had transient asymptomatic arrhythmias. Left and right ventricular function assessed by echocardiogram and postoperative cardiac catheterization were within normal ranges in all but two patients, one with pulmonary artery stenosis and one (Taussig-Bing abnormality with two large ventricular septal defects) with severe pulmonary vascular disease (9.6 units) observed before anatomic repair. The right ventricular pressure at catheterization ranged from 27 to 42 mm Hg in 12 patients and was 55 mm Hg in two. There was no aortic stenosis. Aortic insufficiency was trivial in three patients and mild in one. We conclude that excellent results can be obtained with arterial switch for transposition of the great arteries with or without ventricular septal defect, especially in neonates.     

Repair of persistent truncus arteriosus with interrupted aortic arch.

OBJECTIVE: The aim of our study was to analyse experience with repair of truncus arteriosus with interrupted aortic arch. METHODS: Between 1993 and 2004, eight consecutive patients underwent repair of truncus arteriosus with interrupted aortic arch. The median age was 6.5 days (range 1-85 days) and median weight was 3.2 kg (range 2.6-4.8 kg). Five patients had type A and 3 patients had type B aortic arch interruption. The repair was performed in deep hypothermia with circulatory arrest in 4 patients and isolated selective low-flow perfusion of the head and the heart in the last 4 patients. The repair consisted in aortic arch reconstruction by direct anastomosis between descending and ascending aorta, closure of ventricular septal defect and reconstruction of the right ventricular to pulmonary artery continuity using a valved conduit. RESULTS: One (12.5%) patient died from sepsis and hepato-renal failure 18 days after surgery. Seven (87.5%) patients were followed up for 2.0-11.7 years (median 2.6 years). No patient died after the discharge from hospital. In 4 patients 1-3 reinterventions were required 0.6-10.0 years after repair. Reoperations were performed for conduit obstruction in 2 patients, aortic regurgitation in 2 patients, right pulmonary artery stenosis in 2 patients and airway obstruction in 1 patient. In 2 patients concommitant aortic valve and conduit replacement was required. Balloon angioplasty for aortic arch obstruction was necessary in 1 patient, and for bilateral pulmonary branch stenosis in 1 patient. Five (28.6%) surviving patients are in NYHA class I and 2 (71.4%) patients are in NYHA class II. CONCLUSIONS: Primary repair of persistent truncus arteriosus with interrupted aortic arch can be done with low mortality and good mid-term results. Aortic arch reconstruction in isolated low-flow perfusion of the head and the heart influences favourably the postoperative recovery. The main postoperative problems are associated with conduit obstruction and aortic insufficiency.     

Left-sided lesions after anatomic repair of transposition of the great arteries, ventricular septal defect, and coarctation: surgical factors

OBJECTIVE: This study was undertaken to identify potential anatomic and surgical factors creating left-sided lesions, namely recoarctation of the aorta and neoaortic regurgitation, after anatomic repair of transposition of the great arteries with ventricular septal defect and aortic coarctation. METHODS: From 1983 to September 2002, 109 survivors out of 120 patients were studied. Two-stage repair was performed in 42 patients (group A), and single-stage repair was performed in 67 (groups B and C). Before repair, the diameters of the ascending aorta and main pulmonary artery were measured. In the patients with single-stage repair, coarctation was repaired by extended end-to-end anastomosis in 35 patients (group B) and by pulmonary homograft patch augmentation in 32 patients (group C). The ventricular septal defect was closed through the pulmonary artery in 70 patients and through the right ventricle or atrium in 39 patients. The neoaorto-aortic discrepancy was treated by V-shaped resection of the posterior sinus of Valsalva in 7 cases, pulmonary homograft patch in 32 cases, and anterior splitting of the ascending aorta in all cases. Before discharge from the hospital, neoaortic root and ascending aorta diameters and aortic regurgitation grade were recorded. Neoaortic regurgitation progression and reintervention were the end points of follow-up (97.2 +/- 61.2 months). RESULTS: Early and late survivals were significantly better in group C (P <.001). Risk factors for neoaortic regurgitation at discharge by univariate analysis were single-stage repair (P <.05) and ventricular septal defect closure through the pulmonary artery (P =.0076). On multivariate analysis, the latter was the only risk factor for neoaortic regurgitation at discharge and at last follow-up. Multivariate analysis showed that higher neoaortic root/ascending aorta ratio and ventricular septal defect closure through the pulmonary artery were risk factors for neoaortic regurgitation evolution at last follow-up. There were 29 reinterventions, 19 for recoarctation of the aorta and 10 for neoaortic regurgitation with or without aortic root dilatation. Group B (P <.05), high neoaortic root/ascending aorta ratio (P <.01), and progressive neoaortic regurgitation (P <.05) were risk factors for recoarctation of the aorta. Group A was a risk factor for aortic valve replacement at 10 years (P <.05). CONCLUSION: Neonatal single-stage repair with pulmonary homograft aortic augmentation remains the optimal approach to transposition of the great arteries with ventricular septal defect and aortic coarctation. It provides better early and late survivals and freedoms from left-sided lesions. Avoidance of late recoarctation of the aorta and progressive neoaortic regurgitation requires meticulous closure of the ventricular septal defect and evenly sized reconstruction of the aorta from root to distal arch.     

Pulmonary homograft implantation for ventricular outflow tract reconstruction: early phase results

The pulmonary valve homograft (PH) has been reported to have potential advantages over the aortic valve homograft, including a larger diameter, a thinner wall, and decreased intrinsic calcification. From January 16, 1986, to July 14, 1987, eight consecutive patients underwent repair of congenital cardiac anomalies using a cryopreserved PH. Patients ranged in age from 18 months to 32 years. Diagnoses included tetralogy of Fallot with pulmonary atresia (3 patients); tetralogy with absent pulmonary valve (1 patient); corrected transposition with pulmonic stenosis (1 patient); transposition of the great arteries, ventricular septal defect, and pulmonic stenosis (2 patients); and double-outlet right ventricle with pulmonic stenosis (1 patient). The PH was implanted orthotopically in the patient with absent pulmonary valve, and in the other 7 it was placed as a valved extracardiac conduit. Two of the tetralogy patients with severe bifurcational pulmonary stenosis and another with nonconfluent pulmonary arteries and origin of the left pulmonary artery from a patent ductus arteriosus had their repairs facilitated using the branching pulmonary arterial portion of the PH. There were no hospital or posthospital deaths. Postrepair right ventricular to left ventricular systolic pressure ratios were a mean of 0.35 at 18 hours postoperatively (range, 0.21-0.61). All patients were studied with Doppler and echocardiography after repair. The mean gradient across the PH was 9 mm Hg (range, 2-27 mm Hg), and no pulmonary valve incompetence was present. One patient (12.5%) required reoperation seven months after repair for conduit revision due to compression by the sternum and is now well.(ABSTRACT TRUNCATED AT 250 WORDS)     

完全性大血管错位和右心室双出口肺动脉瓣下室间隔缺损伴主动脉弓病变的一期矫治手术

目的评估一期纠治完全性大血管错位(TGA)和右心室双出口肺动脉瓣下室间隔缺损(Taussig-B ing)伴主动脉弓病变的手术疗效。方法2001年1月—2004年6月对8例伴主动脉弓病变的TGA(3例)和Taussig-B ing(5例)行一期手术治疗。3例TGA中,室间隔完整型1例,伴室间隔缺损2例;主动脉弓病变为7例主动脉缩窄、1例主动脉弓中断。手术年龄1例为8个月,7例为5 d~3个月,平均40 d,体重3.5~6.3 kg,平均(4.3±0.5)kg。均采用胸骨正中切口。手术先在深低温、停循环下矫治主动脉弓病变,然后在深低温、低流量下行大动脉转换术(Sw itch术)。体外循环转流时间107~159 m in,平均(126±23)m in,主动脉阻断时间63~118 m in,平均(92±16)m in,停循环14~45 m in,平均(30±12)m in。结果手术死亡1例,为8个月Taussig-B ing伴主动脉弓发育不良、冠状动脉畸形患儿,术后因低心排血综合征、Ⅲ度房室传导阻滞、肺高压危象死亡;1例3月龄患儿术后5 d喂奶时窒息死亡。6例随访5个月~2年,生长发育良好,1例Taussig-B ing主动脉弓中断出现吻合口狭窄,压差60 mm Hg;2例出现主动脉瓣轻微返流,1例肺动脉瓣轻度返流。结论一期纠治TGA和Taussig-B ing伴主动脉弓病变能取得较好手术效果,手术死亡原因为肺动脉高压和冠状动脉畸形。     

Taussig-Bing畸形合并大动脉侧侧位的外科治疗

目的总结外科手术一期治疗Taussig—Bing畸形合并大动脉侧侧位的临床经验。方法2000年5月～2007年9月手术治疗26例（手术年龄3.1±2．2个月）Taussig—Bing畸形合并大动脉侧侧位患者，其中合并主动脉弓病变13例。25例患者采用动脉转位术（arterial switch operation，ASO），1例采用Kawashima手术治疗。合并主动脉弓病变的矫治方法主要是切除缩窄段后，将胸主动脉与主动脉弓下缘行端端吻合，或与升主动脉行端侧吻合。结果住院死亡3例（11.5％，3／26）。2005年6月以后连续手术治疗的15例Taussig—Bing畸形伴大动脉侧侧位，无住院死亡。随访21例，随访时间1～5年；患者生长发育均明显改善，心脏较术前缩小，肺血明显减少。2例患者因肺动脉瓣上狭窄而再次行手术修补。结论Taussig—Bing畸形合并大动脉侧侧位的病理解剖比较复杂，必须根据不同的解剖条件选择不同的手术方法，才能降低手术死亡率。     

Anatomic correction for complete transposition and double-outlet right ventricle

Between February, 1981, and December, 1984, 30 patients underwent anatomic correction of transposition of the great arteries with intact ventricular septum (n = 8), transposition with ventricular septal defect (n = 15), and double-outlet right ventricle with subpulmonary ventricular septal defect, the Taussig-Bing anomaly (n = 7). At operation, ages ranged from 18 hours to 6 years (mean 11.3 months) and weights ranged from 2.6 to 16.4 kg (mean 6.1 kg). The group with transposition and intact ventricular septum on average was younger (mean 1.2 months) and smaller (mean 3.5 kg) than the other two groups. Associated congenital heart defects were seen in 12 patients, including five with coarctation, three with multiple ventricular septal defects, two with right ventricular hypoplasia, two with juxtaposed atrial appendages, and one each with interrupted aortic arch, Wolff-Parkinson-White syndrome, and left ventricular outflow tract obstruction. All 10 patients who had undergone prior palliative operations had pulmonary artery banding. In addition, four of these patients had coarctation repairs, four had atrial septectomy, and one had systemic/pulmonary shunting. All recognized patterns of coronary anatomy were encountered. The aorta and pulmonary artery were side by side in 14 patients and anteroposterior in 16 patients. The Lecompte maneuver to establish right ventricular-pulmonary arterial continuity was successfully used in 12 of 13 patients with anteroposterior great vessels but in none of those with side-by-side arteries. Seven patients had subvalvular right ventricular outflow tract obstruction, recognized either at operation (five) or postoperatively (two). This was responsible for death in three patients. The 30 day hospital mortalities were as follows: one death (12.5%) in the group with transposition and intact ventricular septum, six deaths (40%) in the group with transposition plus ventricular septal defect, and one death (14.3%) among patients with double-outlet right ventricle and subpulmonary ventricular septal defect; the overall mortality was eight deaths (26.7%). There have been no late deaths (mean follow-up 17.2 months). Ninety-five percent of the survivors are in New York Heart Association Functional Class I. Postoperative catheterization in 13 patients has shown normal left ventricular function, no coronary stenosis, and no aortic incompetence. Sixty-nine percent of these patients had clinically unsuspected gradients across the right ventricular outflow tract, which may be prevented by avoiding the Lecompte maneuver or the use of conduits.