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。     

Primary biventricular repair for interrupted aortic arch with left ventricular outflow tract obstruction and tricuspid valve regurgitation; report of a case

An interrupted aortic arch was diagnosed in a 10-day-old girl weighing 3.3 kg, as was perimembranous ventricular septal defect (VSD) and severe tricuspid valve regurgitation (TR). The subaortic diameter was 3.6 mm and the aortic valve (3.7 mm in diameter) was bicuspid. We chose definitive repair, modified Yasui procedure, because of severe TR and no straddling of mitral valve. In primary biventricular repair, we undertook extended aortic arch anastomosis. Left ventricular outflow tract reconstruction consisted of intracardiac rerouting from the VSD to the pulmonary artery by using expanded-polytetrafluoroethylene (ePTFE) and Damus-Kaye-Stansel (DKS) anastomosis. Right ventricular outflow tract reconstruction was performed by the Rastelli procedure with an ePTFE valved conduit. Moreover, we carried out semicircular annuloplasty for severe TR.     

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.     

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。     

Surgery for transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction: European Congenital Heart Surgeons Association multicentre study

Objectives: Optimal surgical management for patients with transposition of the great arteries (TGA), ventricular septal defect (VSD) and left ventricular outflow obstruction (LVOTO) remains controversial. Although the Rastelli operation has been the most widely performed surgical procedure during the past decades, several studies have shown its suboptimal long-term prognosis. Other operations have been developed to improve results. This study was performed to compare the outcomes of the different surgical approaches for patients with TGA, VSD and LVOTO, as well as to determine risk factors for mortality and re-intervention. Methods: Records from 146 patients undergoing surgery from 1980 to 2008 from eight European hospitals were reviewed. Median age at operation was 21.5 months (range 0.2–165.1 months), and median weight was 10.0 kg (range 2.0–41.0 kg). Surgical procedures involved were the Rastelli procedure (82), arterial (24) and atrial (5) switch operation with relief of LVOTO, Réparation à l’Etage ventriculaire (REV) procedure (7) and the Metras modification (24), as well as the Nikaidoh procedure (4). Results: The overall survival was 88%, 88% and 58% at 1, 10 and 20 years, respectively. The overall event-free survival was 80%, 45% and 26% at 1, 10 and 20 years, respectively. The REV procedure and the Metras modification were found to have the best long-term results in both survival and event-free survival rates. Multivariate analysis revealed year of operation, non-commitment of the VSD and prolonged cardiopulmonary bypass (CPB) time as risk factors for mortality while age at surgery, year of operation and type of corrective surgery were risk factors for re-intervention. Conclusions: Different surgical approaches have been developed for patients with TGA, VSD and LVOTO. The REV procedure and the Metras modification were observed to have favourable long-term results in survival and event-free survival rates. Aortic translocation techniques such as the Nikaidoh procedure seem promising, but further studies will be needed to confirm this in the long term.     

Repair of transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction.

OBJECTIVES: This study was undertaken to compare the outcomes of the Lecompte procedure and Rastelli repair in the transposition of the great arteries (TGA) with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO) and to determine the risk factors associated with unfavorable events. METHODS: Over a 12-year period (April 1990-October 2002), 35 patients underwent complete repair for TGA, VSD, and LVOTO. Twenty-five patients (71%) underwent the Lecompte modification, and mean age and weight were 23.4+/-18.2 months and 10.2+/-3.0 kg. Ten patients (29%) underwent the Rastelli operation, and mean age and weight were 39.1+/-36.1 months and 13.8+/-6.8 kg. RESULTS: One early death (3%) occurred after the Lecompte procedure and no late death. The mean follow-up was 5.9+/-3.8 years. Eight patients in the Rastelli group (80%) underwent a late reoperation for obstruction of the extracardiac conduit, and in four of these patients, a reoperation for LVOTO was concomitantly required. Reoperation was also required in six patients of the Lecompte group (25%); five for right ventricular outflow tract obstruction (RVOTO) including one for LVOTO and two for VSD leakage, and one for mitral regurgitation and left pulmonary artery stenosis. The interval prior to reoperation ranged from 1.6 to 11.1 years, with a mean of 5.7+/-3.1 years. The actuarial figures for freedom from reoperation at 5 and 10 years were 40.0+/-15.5 and 26.7+/-15.0% after the Rastelli operation and 95.7+/-4.3 and 63.5+/-12.6% after the Lecompte procedure (P = 0.02). Multivariate analysis by Cox regression analysis revealed that the risk factors of RVOTO were a younger age at operation, the Rastelli operation, and ductus ligation during the operation. CONCLUSIONS: The Lecompte procedure and Rastelli repair provide satisfactory early and late results. However, substantial late morbidity is more associated with conduit obstruction, and LVOTO in Rastelli repair rather than Lecompte procedure.     

Taussig-Bing anomaly with straddling mitral valve.

Five patients with the left-sided or intermediate type (Lev) of the Taussig-Bing anomaly were found to have an associated straddling of the mitral valve. In four patients the anomaly of the mitral valve was not recognized preoperatively, and all four died postoperatively. The persistent subpulmonic obstruction caused by the abnormal attachment of the anterior mitral leaflet is considered a significant factor in the poor operative outcome. Retrospective study of angiograms in these four patients revealed diagnostic clues of straddling mitral valve which enabled us to diagnose a fifth patient angiographically and confirm the diagnosis by cross-sectional echocardiography. A surgical approach to correct this association of abnormalities is proposed which avoids operating upon the mitral valve. Its function is thereby preserved, yet the hemodynamic problem caused by the straddling is bypassed. The fifth patient in this series was successfully treated by the proposed operative method.     

One stage repair of aortic arch anomalies and intracardiac defects

Between August 1985 and May 1990, 27 neonates and infants underwent combined correction of intracardiac and aortic arch anomalies through a median sternotomy. Coarctation (CoA) was combined with VSD (6), AVSD (2), Taussig-Bing (TB) heart (5), transposition of the great arteries (TGA) (1), TGA + VSD (2), congenitally corrected TGA + VSD (1) and VSD + myxoid stenotic outlet valves (1). Interrupted aortic arch (IAA) was combined with VSD (10) and TB heart (1). Two patients had IAA type B as well as CoA. Age at operation varied from 2 to 243 days with a mean age of 51 days. Twenty patients (70%) were younger than 30 days. One TGA + VSD and all TB hearts had maligned outlet septum and right ventricular outflow tract obstruction (RVOTO). Posterior outlet septum deviation and left ventricular outflow tract obstruction (LVOTO) occurred in 8 patients with malalignment VSD and IAA (7) or CoA (1). Aortic arch reconstruction was performed using extended end-to-end anastomoses. In 3 patients, arch hypoplasia necessitated patch implantation. VSDs were closed through the right atrium unless the anatomy dictated otherwise. One TB heart was reconstructed with a Kawashima procedure. All other TB hearts and all TGAs were corrected with arterial switch operation. Obstructing outlet septum was resected whenever necessary. Follow-up was complete and included echo-Doppler control. Eleven patients had postoperative heart catheterisation. Early mortality was 18.5% (5 patients). Persisting LVOTO or RVOTO was responsible. There was no late mortality. Five patients were reoperated upon: 3 for stenotic anastomoses and 2 for a subaortic membranous stenosis. Successful balloon dilatation of recoarctation was performed four times.(ABSTRACT TRUNCATED AT 250 WORDS)     

Morphology of ventricular septal defects in complete transposition. Surgical implications.

Postmortem examination of 62 hearts with complete transposition (concordant atrioventricular and discordant ventriculoarterial connections) and an accompanying ventricular septal defect was performed to determine the morphologic variability of ventricular septal defects and to explore the surgical implications of these defects. Particular attention was directed toward assessing alignment of the outlet septum relative to the muscular septum. Coronary arterial distributions were also evaluated, but specific patterns of distribution did not correlate with morphology of the defect. Of 49 hearts with a normally aligned outlet septum, there were 24 perimembranous, 21 muscular, and 2 doubly committed and juxtaarterial defects. Two hearts had both perimembranous and muscular defects. Twelve of the 21 muscular defects were "central," being surrounded entirely by muscle and located just below the leaflets of the pulmonary valve, and 9 were located in the inlet or apical trabecular septum. There were 13 hearts with malalignment of the outlet septum, anteriorly in 11 and posteriorly in 2. All with anterior malalignment had a subpulmonary defect that was perimembranous in 7 and muscular in 4. Both defects with posterior malalignment had a subaortic perimembranous defect. Because variations in morphology of a ventricular septal defect have a direct impact on selection of the most suitable surgical repair, specific operative approaches are discussed.     

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.     

完全型大动脉错位伴室间隔缺损、肺动脉狭窄的外科治疗

治疗完全型大动脉错位伴室间隔缺损、肺动脉狭窄(TGA/VSD,PS)的手术方式有Rastelli手术、Lecompte手术、Nikaidoh手术、Yamagishi手术以及动脉转位 左心室流出道扩大术等多种术式,Rastelli手术和Lecompte手术损伤小,但远期并发症多;Nikaidoh手术和Yamagishi手术解剖纠治完全,但手术损伤大;动脉转位术 左心室流出道扩大术适应证相对较狭窄。因此,在TGA/VSD,PS的外科治疗上,应根据患者的自身情况和手术指征采取不同的手术方法。现对上述手术术式的优缺点、适应证、禁忌证、疗效和展望进行综述。     

A new method of closing the ventricular septal defect in corrected transposition of the great arteries

A new method of closing a perimembranous malalignment ventricular septal defect (VSD) in corrected transposition of the great arteries (TGA) of the [S,L,L] type is presented. The method consists of combined approaches to the VSD through both a right atriotomy and an aortotomy without a ventriculotomy. The VSD is patched obliquely from the morphological right ventricular side of the septum, cranially through the aortic valve to the left ventricular side of the septum, caudally through the mitral valve. Although this method has been successfully applied in only one adult patient, some advantages may be expected: (1) prevention of trauma to the His bundle, which runs along the anterosuperior rim of the VSD on the left ventricular side; and (2) prevention of trauma to the tricuspid, mitral, and aortic valves without having to open the ventricles. We believe that this new method warrants a further trial as possibly better for closure of the VSD in corrected TGA of the [S,L,L] type.     

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.     

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.     

Midterm follow-up of arterial switch operation for transposition of the great arteries with intact ventricular septum and left-ventricular outflow tract obstruction

Objective: We report the mid-term follow-up of patients, who underwent arterial switch operation (ASO) for transposition of the great arteries (TGA) with intact ventricular septum and left-ventricular outflow tract obstruction (LVOTO) over a 10-year period from 2000 to 2009. Methods: Thirteen TGA patients (3.9% of our ASO cohort) with intact ventricular septum and LVOTO underwent ASO. LVOTO was defined as pulmonary valve z-score ≤ −2.0 (n = 3) or peak LVOT gradient ≥40 mmHg with (n = 7) or without (n = 3) anatomic subvalvar stenosis on echocardiography. Median age and weight were 14 days (range, 7–130 days) and 3.2 kg (range, 2.1–4.6 kg). The LVOT abnormalities included fibromuscular narrowing (n = 5) and atrioventricular valve-related findings (n = 5). LVOT clearance was achieved by resection of accessory mitral tissue (n = 2) only. Results: Follow-up was 100% complete. There were no early or late deaths. Freedom from re-operation for neo-aortic valve regurgitation and/or LVOTO was 100% at a median follow-up of 38 months (range, 6–115 months). All patients had functional status appropriate for their age. Three patients had mild aortic regurgitation. The median Doppler estimated LVOT systolic gradient was 12 mmHg (range, 0–18 mmHg) for the entire cohort at the latest follow-up. Conclusions: Mid-term outcomes of ASO for a highly selected group of patients with pulmonary valve annulus z-score ≤ −2.0 ≥ −0.4, resectable organic LVOTO, and dynamic peak LVOT gradient ≥40 mmHg remain satisfactory, with a need for long-term follow-up.     

169例动脉调转手术治疗大动脉转位的早、中期结果

目的 探讨大动脉调转手术(ASO)治疗完全性大动脉转位(TGA)的早、中期结果,分析其随访死亡、术后主动脉瓣反流及肺动脉狭窄的危险因素.方法 2004年1月至2007年12月,169例行动脉调转术病儿入选,其中男129例,女40例,平均年龄(11.7±26.3)个月.病儿分两组:Ⅰ组为室间隔完整组(56例),Ⅱ组为室间隔缺损组(113例).所有术后生存病儿均进行超声随访,平均随访时间(27.7±14.6)个月.危险因素采用Logistic回归模型分析.结果 全组住院死亡19例(11.24%),两组间差异无统计学意义.随着整体治疗水平的提高,住院病死率由2004年的16.67%下降到2007年的3.92%.1、3及5年生存率分别为94.00%、91.33%及91.33%,两组间差异无统计学意义.Logistic回归分析发现,ASO术后随访死亡的危险因素为手术年龄大于6个月;术后主动脉瓣反流的危险因素为合并室间隔缺损、年龄大于6个月、术后新主动脉瓣Z值>1;术后肺动脉狭窄的危险因素为手术年龄小于1个月和肺动脉采用补片成形.结论 ASO手术具有良好的早、中期结果,是治疗完全性大动脉转位的理想术式.TGA病儿应该尽早手术治疗,手术年龄大于6个月是随访死亡及主动脉瓣反流的重要危险因素;新主动脉根部与主动脉远端不匹配是术后主动脉瓣反流的危险因素;病儿的生长发育与肺动脉成形材料无生长特性的矛盾是导致术后肺动脉狭窄的危险因素.     

Surgical anatomy of the infundibular septum in transposition of the great arteries with ventricular septal defect

Anatomic variation of the infundibular septum was studied in transposition of the great arteries with ventricular septal defect in 23 hearts and double-outlet right ventricle with anterior position of the aorta in two hearts. Anterior displacement of the infundibular septum (i.e., "false" Taussig-Bing heart) was associated with coarctation or interruption of the aortic arch in 88% of the cases, whereas posterior displacement resulted in subpulmonary narrowing in 100% of the cases. Anterior displacement makes intraventricular rerouting from the left ventricle to the aorta difficult because of a long oblique route. In addition, the right ventricular cavity becomes smaller after closure of the ventricular septal defect. Therefore, arterial switch accompanied with transatrial or transpulmonary closure of the defect without ventriculotomy is recommended. In hearts with posterior displacement of the infundibular septum, the anterosuperior rim of the defect is difficult to approach through the tricuspid valve, and the route from the left ventricle to the aorta is rather straight. Hence, the Rastelli procedure is preferable. In hearts without displacement of the infundibular septum, either arterial or atrial switch with transatrial closure of the ventricular septal defect is applicable.     

右室双出口解剖条件影响矫治手术选择和生存的危险因素分析

目的：通过分析右室双出口的解剖特点、手术选择和结果，以筛选解剖条件中内在的相关规律及与矫治手术死亡相关的危险因素。方法：109例右室双出口病儿，根据不同的室间隔缺损(VSD)位置、大血管位置和并发畸形等解剖特点分类，VSD位置分别与大血管位置和手术方法选择进行相关性检验，Logistic逐步回归筛选与住院死亡相关的危险因素，并建立术前预测死亡概率的模型。结果：主、肺动脉位置为侧侧位或主动脉右前斜位，VSD大多为主动脉下，手术方法以心室内矫治(IVR)为主；大血管位置为前后位，VSD大多为肺动脉瓣下，手术方法以Rastelli和大动脉转位术(ASO)为主。主动脉右前斜位、主动脉瓣下VSD的相对危险度小于1，主动脉左前斜位、VSD远离大动脉开口及左室发育不良等解剖条件的相对危险度大于1，Logistic回归方程组内考核的符合率为80．7％。结论：VSD位置和大血管位置及手术方法选择之间有显著的相关性，主动脉右前斜位、左前斜位、主动脉下VSD或VSD远离大动脉开口及左室发育不良等解剖条件对住院死亡有显著影响。     

Surgical management of double-outlet right ventricle with subaortic ventricular septal defect

Double-outlet right ventricle (DORV) and subaortic ventricular septal defect (VSD) is defined anatomically as a defect where the entire pulmonary trunk and at least half of the aorta arises from the right ventricle (RV) and the VSD is most closely aligned with the aorta. The surgical management of DORV and subaortic VSD usually results in a 2 ventricle repair where the left ventricular outflow is diverted via the VSD to the aorta. Pulmonary and/or subpulmonary obstruction is found in a large portion of these patients and requires relief at the time of repair. Resection of subpulmonary muscular obstruction with right ventricular outflow augmentation with a patch is required in most to eliminate the obstruction. Important anatomic features to consider in constructing the left ventricular outflow tunnel include the prominence of the conal septum, tricuspid valve attachments to conal septum, and the distance between the pulmonary and tricuspid valves. Operative mortality is low and long-term complications and need for reoperation is uncommon. This report describes the surgically relevant anatomic features and operative techniques. Copyright © 2000 by W.B. Saunders Company