Repair of transposition of the great arteries with intact ventricular septum and left ventricular outflow tract obstruction

Repair of transposition of the great arteries in patients with intact ventricular septum and fixed left ventricular outflow tract obstruction has been restricted to atrial baffle procedures, with or without attempts to relieve or bypass the left ventricular outflow obstruction. However, the suboptimal results of these procedures, coupled with excellent functional results with the arterial switch operation in patients without obstruction, has made anatomic correction the goal in repairing these anomalies. We report a technique for the anatomic correction of transposition of the great arteries, intact ventricular septum, and fixed left ventricular outflow tract obstruction. Its consideration in these difficult cases is advocated.     

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.     

Transposition of the great arteries, ventricular septal defect, and pulmonary outflow tract obstruction. Rastelli or Lecompte procedure?

During a 10-year period (1980 to 1990), 62 patients underwent complete repair for transposition of the great arteries, ventricular septal defect, and pulmonary outflow tract obstruction. Twenty-two patients (35%) (mean age 8.1 +/- 7.2 years) underwent the Rastelli operation: The ventricular septal defect was enlarged anteriorly in eight patients, and right ventricular-pulmonary artery continuity was established with an extracardiac valved (9/22) or nonvalved (13/22) conduit. Forty patients (65%) (mean age 3.3 +/- 3.2 years) underwent the Lecompte modifications: The conal septum was extensively excised when present (30/40), anterior translocation of the pulmonary bifurcation was performed in 32 patients, and right ventricular-pulmonary artery continuity was established by direct anastomosis without a prosthetic conduit. There were seven early deaths (11%; 70% confidence limits, 7% to 17%): two after the Rastelli procedure (9%; 70% confidence limits, 3% to 20%) and five after the Lecompte operation (12.5%; 70% confidence limits, 7% to 20%). Four patients were lost to follow-up, yielding a 93% complete follow-up (mean follow-up 55 months). There were two late deaths (one in each group). Actuarial probability of survival (+/- standard error) at 5 years was 83% +/- 9% after the Rastelli operation and 84% +/- 6% after the Lecompte procedure. All long-term survivors (except one in the Rastelli group) were in functional class I. Five patients in the Rastelli group underwent late reoperation for obstruction of the extracardiac conduit (28%; 70% confidence limits, 16% to 42%). Three late reoperations (10%; 70% confidence limits, 4% to 19%) were required after the Lecompte operation (one for residual ventricular septal defect and two for residual pulmonary outflow tract obstruction). At most recent examination, residual pulmonary outflow tract obstruction was present in seven patients of the Rastelli group (39%; 70% confidence limits, 26% to 53%) and in six patients of the Lecompte group (19%; 70% confidence limits, 12% to 29%). The combined likelihood of reoperation for pulmonary outflow tract obstruction and residual pulmonary outflow tract obstruction was significantly higher in the Rastelli group (67% versus 26%; p = 0.005). Both procedures provide satisfactory early and late results. The Lecompte operation allows complete repair in infancy, is feasible in patients with anatomic contraindications to the Rastelli operation, and may reduce the need for reoperation and the prevalence of residual pulmonary outflow tract obstruction.     

Complete transposition of the great arteries with intact ventricular septum and left ventricular outflow tract obstruction. Surgical management and anatomic considerations.

Surgical management of patients with complete transposition and intact ventricular septum may become difficult in the presence of left ventricular outflow tract obstruction. A Mustard operation and direct resection of the obstruction through the pulmonary artery has been the treatment of choice for this combination. Our study of the structure of the left ventricular outflow tract in four specimens with the anatomic findings of complete transposition, intact ventricular septum, and subpulmonary stenosis suggests that direct resection of the stenosis through the pulmonary artery can seldom be adequate without major risk of damaging either the mitral valve or the conduction tissue. An alternative procedure, namely, a combined Mustard operation and insertion of an external conduit from the left ventricle to the main pulmonary artery, has been employed in the management of six patients with this combination of lesions. One early and one late death occurred. Postoperative cardiac catheterization performed in all of the survivors before discharge from the hospital showed good relief of the stenosis and no significant gradient across the conduit.     

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.     

Apico-pulmonary artery conduit repair of congenitally corrected transposition of the great arteries with ventricular septal defect and pulmonary outflow tract obstruction: a 10-year follow-up

BACKGROUND: In conventional repair of the congenitally corrected transpositions of the great arteries associated with ventricular septal defect and pulmonary outflow tract obstruction, the placement of the left ventricle-pulmonary artery conduit is at risk owing to probable compression by the sternum, heart block, or injury to the mitral anterior papillary muscle. Apical placement of the left ventriculotomy for the inflow conduit rather than in the midportion or base placement may avoid these complications, although this results in a long and winding extracardiac conduit that may be short-lived because of the proliferation of pseudointima. METHODS: Between 1985 and 1990, a nonvalved Dacron woven-fabric graft conduit was placed between the left ventricular apex and pulmonary artery in 5 patients (mean age, 6.2 +/- 1.7 years) who were then followed for at least 10 years. RESULTS: No iatrogenic heart blocks or mitral regurgitation developed. All patients were complaint-free during the follow-up period, although 1 patient who was clinically well died suddenly in the 10th follow-up year. Cardiac catheterization in the 10th follow-up year indicated a pressure gradient of 21 +/- 6 mm Hg across the conduit, and angiography revealed that the conduit diameter was 91% +/- 6% of the original conduit diameter. CONCLUSIONS: The reportedly poor early and late outcomes that occur after a conventional repair of congenitally corrected transpositions of the great arteries associated with ventricular septal defect and pulmonary outflow tract obstruction, which places an extracardiac conduit between the left ventricle and the pulmonary artery, may be partially neutralized by relocating the inflow position to the apex.     

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.     

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.     

Neonatal anatomic repair of transposition of the great arteries and ventricular septal defect

From January 1985 to March 1992, 64 consecutive neonates with transposition of the great arteries (TGA) and ventricular septal defect (VSD) underwent an arterial switch operation and VSD closure. The mean age at operation was 18.5 ± 12 days and the mean weight was 3.3 kg. Seventeen patients had an associated aortic coarctation, of whom 15 underwent single-stage repair through median sternotomy. Coronary artery distribution was: type A: 45 patients; type B: 2; type D: 11 and type E: 6 patients. The location of the VSD was perimenbranous in 42 patients, trabecular in 13, infundibular in 5, and 4 presented with the Taussig Bing heart anomaly. The hospital mortality was 9.3% (n = 6). There were four late deaths (one TGA-VSD and three TGA-VSD and coarctation). Nine patients required reoperation. The mean follow-up of all survivors was 36 ± 19 months. They were in NYHA class I without medication. Six patients developed mild-to-moderate aortic insufficiency. The actuarial survival and freedom from reoperation at 5 years were 81.06% and 84.6%, respectively. We conclude that neonatal anatomic repair of TGA and VSD offers good medium-term results and avoids iterative operations.     

Repair of complex left ventricular outflow tract obstruction in children

Background Complex obstruction of the left ventricular outflow tract (LVOTO) in children may be treated with surgical procedures like the Ross–Konno or Koncz–Konno procedures, or modifications thereof. We present our results from the last 10 years.

Material and method During the period from December 1991 to April 2002, 17 patients/children with complex LVOTO were operated on. Patients treated with balloon valvuloplasty, open commisurotomy or simple valve replacement are excluded. The procedures performed were: Koncz–Konno (KK) or modified KK (n=10), Ross–Konno (RK) (n=2) and Ross (RO) (n=5). Concomitant procedures included mitral valve replacement (n=1), aortic arc plasty (n=1), subvalvular myotomi (n=1), resection of subvalvular membrane (n=1) and aorto-coronary bypass (n=1). Patients’ ages ranged from 1 to 178 months, median 71 months. Body weights ranged from 3.8 to 50.0 kg, median 19.4 kg. Eight cases were redo procedures.

Results Three early deaths occurred. One patient in the KK group, one in the modified KK group and one in the RK group. One patient died 8 years postoperatively of unknown reasons; no autopsy was performed. The rest of our patients who survived the initial major surgical treatment show normal physical performance and their quality of life seems to be good.

Conclusion Patients with complex LVOTO can be operated with biventricular repair in selected cases. Early and late mortality are acceptable.     

Repair of complex left ventricular outflow tract obstruction in children

BACKGROUND: Complex obstruction of the left ventricular outflow tract (LVOTO) in children may be treated with surgical procedures like the Ross-Konno or Koncz-Konno procedures, or modifications thereof. We present our results from the last 10 years. MATERIAL AND METHOD: During the period from December 1991 to April 2002, 17 patients/children with complex LVOTO were operated on. Patients treated with balloon valvuloplasty, open commisurotomy or simple valve replacement are excluded. The procedures performed were: Koncz-Konno (KK) or modified KK (n = 10), Ross-Konno (RK) (n = 2) and Ross (RO) (n = 5). Concomitant procedures included mitral valve replacement (n = 1), aortic arc plasty (n = 1), subvalvular myotomi (n = 1), resection of subvalvular membrane (n = 1) and aorto-coronary bypass (n = 1). Patients' ages ranged from 1 to 178 months, median 71 months. Body weights ranged from 3.8 to 50.0 kg, median 19.4 kg. Eight cases were redo procedures. RESULTS: Three early deaths occurred. One patient in the KK group, one in the modified KK group and one in the RK group. One patient died 8 years postoperatively of unknown reasons; no autopsy was performed. The rest of our patients who survived the initial major surgical treatment show normal physical performance and their quality of life seems to be good. CONCLUSION: Patients with complex LVOTO can be operated with biventricular repair in selected cases. Early and late mortality are acceptable.