The optimal procedure for the great arteries and left ventricular outflow tract obstruction. An anatomical study.

OBJECTIVE: To describe the optimal surgical strategy in heart specimens with transposition of the great arteries (TGA) and left ventricular outflow tract obstruction (LVOTO). METHODS: Thirty-three specimens with LVOTO were selected: TGA with intact ventricular septum (TGA/IVS) (10), TGA/VSD (21), and Taussig-Bing (2). RESULTS: LVOTO in TGA/IVS consisted of combinations of bicuspid pulmonary valve (four), subpulmonary fibrous ridge (four), obstructive muscular conus (two) and bulging muscular septum (four). Arterial switch operation (ASO) with LVOTO resection/valvotomy was feasible in nine hearts. Obstructive anterior papillary muscle prohibited LVOTO relief in one specimen. In TGA/VSD and Taussig-Bing LVOTO consisted of combinations of bicuspid (nine) or unicommissural (one) pulmonary valve, fibrous ridge (three), obstructive muscular conus (five), malaligned outlet septum (six), accessory mitral valve tissue (two), straddling mitral valve (two) and anterior mitral valve rotation (four). VSDs were subpulmonary in 13 (9 perimembranous, 4 muscular), subaortic in 3 (2 perimembranous, 1 anterior muscular), doubly committed in 2, inlet in 3 (2 perimembranous, 1 muscular), non-committed and anterior in 1, and finally 1 VSD extended both into inlet and subpulmonary outlet septum. LVOTO resection and ASO with VSD closure was possible in 10. In six specimens, both a Rastelli and a Nikaidoh operation were feasible. For two hearts, a Nikaidoh procedure was the only option, while Rastelli was considered optimal in another specimen. Mitral valve anomalies prevented LVOTO relief in four, only permitting for Senning/VSD closure (one) or univentricular palliation (three). CONCLUSIONS: LVOTO resection and pulmonary valvotomy frequently permits an ASO. Inlet VSD, impossibility of VSD enlargement, straddling mitral valve, distant aorta and small right ventricle make the Nikaidoh procedure the best option. Mitral anomalies preventing LVOTO relief can make biventricular repair impossible.     

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.     

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。     

阜外医院71例Rastelli手术的近中期结果

目的总结分析阜外医院Rastelli手术的近中期结果。方法2010年5月至2017年3月阜外医院完成71例Rastelli手术治疗大动脉转位(TGA)或者右心室双出口(DORV)合并室间隔缺损(VSD)和肺动脉狭窄患儿,男48例,女23例;手术时年龄(4.7±2.7)岁,其中10例完全型大动脉转位;27例DORV,34例矫正型TGA(CTGA)。30例有手术史,其中Blalock-Taussig分流13例,双向Glenn手术17例。31例同时扩大室间隔缺损。外管道使用同种带瓣管道9例,牛颈静脉管道56例,自制Gore-Tex外管道6例;管道直径(17.9±3.3)mm。结果本组主动脉阻断(132.0±71.1)min,体外循环(209.0±83.4)min。机械通气时间(102.6±81.7)h。术后住院(13.6±12.8)天。早期死亡1例(1.4%)。早期并发症12例(16.9%),其中因Ⅲ度房室传导阻滞安装永久起搏器4例,心包积液开窗3例,延迟关胸3例,二次开胸2例。术后随访4个月~6.8年。1、5年生存率分别为97.2%,97.2%;1、5年免除右心室流出道狭窄(RVOTO)率分别为98.6%,84.1%;1、5年免除干预率分别为98.6%,90.0%。随访期间置换外管道1例,介入球囊扩张7例10次。所有患儿最后一次随访均未见左心室流出道狭窄,左心室到主动脉压差(10.5±8.8)mmHg。结论Rastelli手术适用于部分DORV、CTGA、TGA合并VSD和严重的肺动脉狭窄或肺动脉闭锁患儿,早期死亡比例低,中期效果好,但是远期常需要再手术,尤其是置换外管道。     

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.     

Réparation à l'Etage Ventriculaire (REV Procedure): Not a Rastelli Procedure Without Conduit

Since the introduction of the REV procedure in 1980, this operation has become our procedure of choice for patients with transposition of the great arteries, VSD, and POT obstruction. The classical Rastelli procedure is indicated only in patients in whom the presence of a well-functioning pulmonary valve is deemed necessary (i.e., hypoplastic right ventricle, inadequate pulmonary arterial bed). Compared with the Rastelli operation, the REV procedure may have definite advantages:

1. The REV operation creates a straight, short left ventricular-aortic tunnel, provides proper alignment of the aorta with the left ventricular chamber, and practically eliminates the risk of late subaortic stenosis.

2. Complete repair is feasible in patients in whom a Rastelli procedure would be contraindicated because of unfavorable intracardiac anatomy (i.e., restrictive VSD, abnormal attachment of tricuspid chordae on the conal septum).

3. The absence of an extracardiac conduit to restore the right ventricular-pulmonary artery continuity reduces the need for reoperation due to recurrent POT obstruction. In most patients, the long-term outcome of the reconstructed right ventricular outflow tract may be similar to that of a repaired tetralogy of Fallot. However, the need to relieve right ventricular outflow tract obstruction, although reduced in comparison with the Rastelli procedure, is not eliminated completely.

4. Because of its feasibility in most intracardiac situations and because it obviates the need for an extracardiac conduit, the REV procedure can be performed in infants and small children, thus avoiding, in most patients, an initial palliative step.

Outcomes of 829 neonates with complete transposition of the great arteries 12-17 years after repair.

OBJECTIVE: Between 1985 and 1989, the surgical management of neonates with complete transposition (TGA) underwent a transition from atrial to arterial repair. We sought to examine the intermediate outcomes and their associated risk factors in neonates repaired during the era of transition. PATIENTS AND METHODS: Twenty-four institutions entered 829 neonates age less than 15 days in a prospective study. Diagnosis was simple TGA (n=631), TGA with ventricular septal defect (VSD) (n=167), TGA with VSD and pulmonary stenosis (TGA/VSD/PS) (n=30), or TGA with PS (n=1). Repair was by arterial switch (n=516), atrial repair (Senning=175, Mustard=110) or Rastelli (n=28). Time-related events were analysed by parametric hazard function modeling and incremental risk factors for mortality, re-intervention, and late functional assessment were sought. RESULTS: Survival estimates at 6 months, 5, 10, and 15 years are 85, 83, 83, and 81%, respectively. The hazard function for death after repair has two phases: an early rapidly declining phase and an ongoing constant one. Constant phase mortality is less likely after the arterial switch operation and in children with simple TGA. During follow up, at least one re-intervention was required in 167 children (pacemaker, n=35; percutaneous intervention, n=32; baffle re-intervention, n=27; re-operation, n=125). Freedom from re-intervention at 6 months, 5, 10 and 15 years is 93, 82, 77, and 76%, respectively. Of survivors, 87% have been followed up to the last 3 years, including an assessment of functional ability of 562 children (83%). Functional class 15 years after repair is class I in 76%, II in 22%, III in 2%. The proportion in functional class I decreased over time. Psychosocial deficits, especially learning disorders are prevalent. CONCLUSIONS: Survival 15 years after TGA repair is good with most children functioning well, and results are best after an arterial switch operation. There is an ongoing risk of death that is less after the arterial switch operation. With the exception of Rastelli patients, the likelihood of survivors needing re-intervention after 5 years is low. There is need for improved neurodevelopmental outcomes.     

Successful biventricular repair after initial Norwood operation for interrupted aortic arch with severe left ventricular outflow tract obstruction

Background. Management of newborns with interrupted aortic arch (IAA) remains challenging. Associated severe left ventricular outflow tract obstruction (LVOTO) have often led to increased mortality with neonatal biventricular repair. We review our experience with an alternative approach for this complex surgical problem.

Methods. From May 1991 to June 1999, 28 neonates were treated for IAA. Thirteen of 28 neonates (46%) had type B IAA, ventricular septal defect (VSD) and severe LVOTO (Z value –2 to –7; mean –5 ± 1.7). Mean age was 8 days (3 to 23 days old) with average weight of 3.3 kg (2.4 to 4.2 kg). Eight of 13 (62%) had anomalous right subclavian artery. Ten of 13 (77%) had thymic aplasia and chromosome 22 region q11 deletion. All 13 patients were treated initially with a modified Norwood procedure.

Results. There were no perioperative deaths. Complications included 2 patients with recurrent arch stenosis treated with balloon dilatation. Two patients had systemic arterial shunt revision. Follow-up ranged from 2 to 99 months old (mean 39 months). There were 2 late deaths unrelated to any operation. Nine of 12 patients had a second stage palliation consisting of a bidirectional Glenn shunt. Six patients went on to have biventricular repairs (3 Ross-Konno, 2 Rastelli, 1 VSD closure with LVOT resection). One patient had a modified Fontan operation and 5 patients are awaiting potential biventricular repair.

Conclusions. Children with IAA and severe LVOTO may be managed by initial Norwood palliation with an excellent outcome likely. This initial “univentricular” approach has enabled eventual successful biventricular repair despite severe LVOTO.     

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.     

完全性大动脉转位的个体化外科治疗

目的 研究不同类型完伞性大动脉转位(TGA)的个体化外科治疗策略及其效果.方法 1998年3月至2009年10月,共收治各类TGA患者127例(158例次),男性97例,女性30例.年龄生后4 h～17岁,平均(25±37)个月,其中<3个月56例66例次;体质量2.7～47.5 kg,平均(8±8)kg.初期手术行Glenn手术14例(其中3例行双侧Glenn手术),主-肺动脉分流术14例,肺动脉环缩术8例,房间隔缺损扩大+肺动脉环缩+主-肺动脉分流术15例.终期手术包括Seaning手术5例,一期Switch手术32例,二期Switch手术11例,Switch手术+室间隔缺损修补术20例,Switch手术+室间隔缺损镶嵌手术1例,Nikaidoh手术3例,Rastelli手术13例,Fontan手术18例,心内隧道等其他手术4例.结果 20例患者接受初期手术后现正在等待或已放弃二期手术,21例患者接受2期手术,5例接受3次及以上手术.66例次患者术后延迟关胸.采用腹膜透析9例,呼吸机使用时间2 h～16 d.全组早期死亡12例(病死率9.4%),死因包括术后低心排出最5例,肺动脉高压危象2例,术中出血2例,冠状动脉畸形1例,感染合并自发性肝破裂1例,肾功能衰竭1例.109例患者术后随访1个月～12年,6例失访,远期2例死亡.随访患者中,10例有不同程度的并发症,3例已再次手术,随访效果好.余存活病例,心功能正常,生长发育良好.结论 根据TGA患者解剖条件,采用个体化治疗策略,制定不同的手术方案,可以明显提高患者的手术成功率和远期生存率.     

Surgical problems in d-TGA with VSD and PS associated with insertion of tricuspid valve chordae to the infundibular septum--the Rastelli operation by translocation of the infundibular septum

Twelve (37.5%) out of 32 patients who underwent surgical repair of complete transposition of the great arteries (d-TGA) with ventricular septal defect (VSD) and pulmonary stenosis (PS) from 1978 to 1987 had insertion of tricuspid valve (TV) chordae to the infundibular septum, i.e. superior margin of the VSD, which precluded the ordinary Rastelli type operation. Of these 12 patients early 5 patients (ages: 2-11 mean 7 years) underwent atrial switch operations with VSD closure and pulmonary valvotomy with or without fibromyotomy, and recent 6 patients (age: 4-8, mean 6 years) underwent the Rastelli operation (3 cases) or REV (3) by translocating the infundibular septum with TV chordae to the right ventricular side of the internal conduit. None died in hospital in the former group and 1 in the latter. Pulmonary/systemic ventricular peak pressure ration (Ppv/sv) was 0.75 +/- 0.22 (mean +/- standard deviation) in the atrial switch group v. 0.49 +/- 0.08 in the Rastelli/REV group 1 month postoperatively. The higher Ppv/rv in the atrial switch group was attributed to the pressure gradient across the pulmonary outflow tract, which was 37.8 +/- 16.6 mmHg in the atrial switch v. 16.7 +/- 4.2 mmHg in the Rastelli/REV (p less than 0.05). No pressure gradient was demonstrated between left ventricle (LV) and aorta in the both groups. Pulmonary wedge pressure was higher in the atrial switch group (13.8 +/- 1.9 mmHg) than in the Rastelli/REV (10.0 +/- 2.7 mmHg) (p less than 0.05). Systemic atrioventricular (AV) valve regurgitation was noted in 2 of the atrial switch group but none in the Rastelli/REV.(ABSTRACT TRUNCATED AT 250 WORDS)     

Successful Rastelli Operation in Infant with Complete Transposition of Great Arteries,Levoposition of Aorta,and Subaortic Ventricular Septal Defect

A 10-month-old infant with complete transposition of the great arteries (TGA) {S, D, L} and large ventricular septal defect (VSD) without left ventricular outflow tract obstruction underwent successful Rastelli operation utilizing a 16 mm Hancock graft. The VSD was subaortic, and an internal conduit was placed easily. Postoperative hemodynamic conditions were satisfactory, and the patient was doing well eighteen months after operation. The feasibility of primary Rastelli operation for TGA and VSD in young infants is described.     

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)     

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.     

保留肺动脉瓣的改良REV手术治疗复杂型大动脉转位——附3例报道及文献综述

目的 介绍一种改良REV手术治疗合并室间隔缺损（VSD）、肺动脉瓣狭窄（PS）的复杂型大动脉转位或右心室双出口（DORV）。方法 2005年9月～2006年2月我院收治3例复杂型先天性心脏病患者，在经典REV手术的基础上，我们改良了REV手术，保留了肺动脉瓣和瓣环进行手术治疗。结果 2例痊愈，1例同时用体外膜式氧合器（ECMO）进行抢救后死亡。痊愈患者分别随访4个月和1个月，超声心动图检查提示肺动脉瓣跨瓣压差分别为15mmHg和5mmHg，结果满意。结论 保留肺动脉瓣的改良REV手术对治疗合并VSD、PS的复杂大动脉转位是一种较为理想的手术方法。     

Primary definitive repair of interrupted aortic arch with ventricular septal defect

The optimal surgical management (primary or staged repair) of interrupted aortic arch (IAA) with ventricular septal defect (VSD) remains to be determined. A consecutive series of 14 neonates, aged 3-18 days (mean: 10 +/- 6 days) underwent primary complete repair. Mean weight was 3.3 +/- 0.4 kg. Eleven patients had IAA type B, 2 had type A and 1 had type C. Six infants had the Di George syndrome. Preoperative management (mean: 5 +/- 4 days) included prostaglandin E1 (14/14), intubation and ventilation (13/14), and inotropic support (11/14). Surgery was performed under deep hypothermia and circulatory arrest and involved resection of all ductal tissue, direct end-to-side aortic arch anastomosis and patch closure of the VSD. There were 2 early deaths (14%, 70% CL: 5%-31%): low cardiac output (1), residual VSD (1). Four patients (33%, 70% CL: 13%-52%) underwent reoperation for recurrent aortic obstruction (3 patients, 1 death) or left ventricular outflow tract obstruction (LVOTO) (1 patient). The results improved with time: no death and no recurrent aortic obstruction in the last 8 patients. At last follow-up (11 patient, mean follow-up = 24 +/- 9 months), all patients were free of cardiac symptoms; none had persistent aortic obstruction; 4 had LVOTO (gradient greater than 20 mm Hg) and 1 (with the Di George syndrome) had severe mental disorders. Primary complete repair provides satisfactory results in most infants born with IAA and VSD. An adequate direct aortic arch anastomosis should entail a low risk of recurrent obstruction. LVOTO develops in many cases and may require further surgery.     

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.     

Closure of ventricular septal defect of interrupted aortic arch with left ventricular outflow obstruction]

Leftward shift of the infundibular septum (IS) in interrupted aortic arch (IAA) with ventricular septal defect (VSD) often develops significant left ventricular outflow obstruction (LVOTO). Seven-day-old boy with 2.6 kg body weight underwent the two-stage operation for this anomaly. The aortic arch was interrupted between the left common carotid and the left subclavian artery. At the first stage, a 5 mm GORE-TEX graft was used to connect the interrupted arch, and pulmonary artery banding was performed. In closure of VSD at the second stage, IS was penetrated by stitches for the VSD patch to left ventricular outflow tract. IS with leftward shift could be pulled toward right ventricular side with patch fixation and LVOTO was prevented by this method.     

Modified Rastelli using an autograft: A new concept for correction of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction (with an extension to other congenital heart defects)

We describe in this article a new approach in transposition of the great arteries (TGA), ventricular septal defect (VSD), and left ventricular outflow tract obstruction (LVOTO). It consists of an operation combining the intracardiac infundibular resection of the Lecompte procedure to allow a direct VSD-aorta connection and the right ventricle-pulmonary artery (RV-PA) connection with a segment of tubular aortic autograft, (with or without an additional monocusp patch). Thus, a viable conduit, capable of growth, is interposed between RV and PA. We have performed this procedure in 19 patients (age, 2 months to 11 years), 6 being under 1 year. All patients are doing well after a maximum follow-up of 72 months, exhibiting growth of the conduit and satisfactory RV pressures. This new approach allows primary correction even in the young and can be extended, we think, for other defects with pulmonary stenosis or atresia. It shares, however, with many other procedures, the valveless nature of the repair with the potential late need for valve replacement surgery. Copyright © 2000 by W.B. Saunders Company