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This review describes the different surgical options for transposition of the great arteries, ventricular septal defect (VSD), and left ventricular outflow tract obstruction. When the pulmonary valve can be used, an arterial switch operation with VSD closure and resection of pulmonary stenosis may be possible. This is not the scope of our review: we focus on the Rastelli, REV (Réparation à l'Etage Ventriculaire), and Nikaidoh techniques, and we also describe the “en bloc rotation” technique. Each of these procedures has a different history, and these techniques have not been used uniformly around the world. We describe the advantages and disadvantages of each technique together with their outcomes as reported in the literature. Some forms of transposition of the great arteries, VSD, and left ventricular outflow tract obstruction can only be corrected by the Nikaidoh operation, although this operation can be definitely contraindicated in other instances. Surgical eras and length of follow-up are not the same for all procedures, and there has been surgical bias in choosing 1 technique over another. This makes comparison between techniques difficult, although certain trends are observed.
Different procedures can be used to repair transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction. Rastelli, REV (Réparation à l'Etage Ventriculaire), Nikaidoh, and “en bloc rotation” techniques are all described with their respective history, advantages, and drawbacks.
Introduction
Transposition of the great arteries (TGA), ventricular septal defect (VSD), and left ventricular outflow tract obstruction (LVOTO) are rare forms of congenital heart disease. In TGA, VSD, and LVOTO, the position of the great arteries and the coronary anatomy can vary. VSDs may be committed or noncommitted and can be small or large. LVOTO can vary greatly.
When the pulmonary valve is big enough and functional, an arterial switch operation with VSD closure and LVOTO resection is possible. This is not the subject of this review. With pulmonary valve hypoplasia or severe valvular dysfunction, the arterial switch is no longer an option. The left ventricle (LV) must be either tunneled to the aorta, or the aorta must be translocated to the LVOT. The Rastelli operation was the first procedure (1968) to correct TGA, VSD, and LVOTO, with the LV as systemic ventricle.
Aortic translocation and biventricular outflow tract reconstruction. A new surgical repair for transposition of the great arteries associated with ventricular septal defect and pulmonary stenosis.
Aortic root translocation plus arterial switch for transposition of the great arteries with left ventricular outflow tract obstruction: intermediate-term results.
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).
En bloc rotation of the truncus arteriosus—an option for anatomic repair of transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction.
Minor modifications of these operations have been published but will not be discussed.
Surgical Options
Rastelli Operation
Before the Rastelli operation, TGA, VSD, and LVOTO were treated by Senning or Mustard procedure and VSD closure. If needed, resection of LVOTO was performed. With the Rastelli operation, however, the LV remains in the systemic circulation. The LV to aortic tunnel is made through a right ventriculotomy that is also used to connect the right ventricle (RV) to the pulmonary arteries with a valved conduit. This conduit is extracardiac and is usually placed leftward of the ascending aorta.
REV Operation
Two essential features of REV are resection of the infundibular septum and direct connection of the pulmonary trunk to the RV. Even when the VSD is big, the conal septum must be resected as much as possible. A Lecompte maneuver is part of the REV procedure. Resection of the outlet septum will make the LV to aorta connection much straighter than in the Rastelli operation. In the Metras modification, a tubular segment of autologous ascending aorta is used to construct the RV to pulmonary artery (PA) connection. A Lecompte maneuver is not used here, and the connection is placed left- or right-sided to the aorta. The intracardiac tunnel is made in a similar fashion as in the REV operation, with resection of the conal septum.
Posterior Aortic Translocation and “En Bloc Rotation” Techniques
In posterior aortic translocation (Nikaidoh-Bex operation), the aorta is detached from the RV and translocated posteriorly onto the LVOT. An advantage is the straight-lined connection between LV and aorta, much less reduction of RV volume, and an RV to PA conduit that is orthotopically placed. The superiority of posterior aortic translocation will be questionable when the LVOT is very small as this limits the distance to which the aorta can be moved backward.
The ascending aorta can be transected slightly above or at the level of the sinotubular junction. This transection is always necessary when the aortic root will be rotated; in other instances, there is no need for aortic transection. Aortic root detachment can be complete or incomplete. When detachment is incomplete, the aorta remains attached under the left coronary artery (LCA) and is rotated clockwise into the LVOT (Fig. 1). The pulmonary valve annulus and the infundibular septum are transected so that the LVOT opens widely. If necessary, subpulmonary obstructive tissue can be further resected. This technique was described by Nikaidoh in 1984.
Aortic translocation and biventricular outflow tract reconstruction. A new surgical repair for transposition of the great arteries associated with ventricular septal defect and pulmonary stenosis.
In our experience, coronary artery detachment and relocation is not necessary; the LCA can remain in place, whereas the right coronary artery (RCA) needs to be mobilized sufficiently to allow (clockwise) rotation of the aortic root. Contrary to our own experience, Nikaidoh found that partial detachment resulted in more patients needing relocation of the RCA more anteriorly than when the aortic root was fully mobilized.
Complete mobilization of the aortic root is the current choice. Complete detachment can be used with or without rotation of the aortic root and with or without detachment and relocation of the coronary arteries.
Figure 1Partial detachment of the aortic root. The area under the left coronary artery remains attached to the right ventricle.
Different strategies to preserve aortic valve function have been reported: operation without cross-clamping the aorta, administration of saline into the aortic root via the cardioplegia needle, and fibrillating heart technique; all these techniques aim to preserve aortic root integrity and prevent distortion of the valve. When the aorta is transected, preserve aortic root integrity is no longer possible.
Our approach to the Nikaidoh operation has always been to leave the aorta intact and partially attached under the LCA.
This facilitates posterior translocation and preserves aortic valve function. To ensure that aortic root geometry is not disturbed, we use a continuous saline flush through the cardioplegia needle to keep the aortic valve closed. There is no need for aortic transection, Lecompte maneuver, or coronary detachment. Constructing the RV-PA conduit (bovine jugular vein conduit) such that it wraps around the aorta does not result in dysfunction and has proved to be a satisfactory way to reconstruct the right ventricular outflow tract (RVOT). The graft can be placed to the right or to the left of the aorta.
More radical techniques have been reported by Yamagishi and by Mair: an autologous half-turned truncal block that involves both semilunar valves is fully detached from the heart and turned 180° before it is re-inserted with the aortic valve on top of the LVOT and the pulmonary valve over the RVOT.
En bloc rotation of the truncus arteriosus—an option for anatomic repair of transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction.
The “en bloc rotation” technique provides sufficient length of the autologous pulmonary posterior wall for direct anastomosis by itself. The risk of late right ventricular outflow tract obstruction (RVOTO) is thought to be reduced by elimination of the extracardiac conduit and by having a fully tubular pulmonary trunk or at least a posterior wall of native tissue.
RVOT reconstruction techniques vary greatly in the operations where posterior aortic translocation is used. A wrap-around pericardial patch technique can be used where the anterior wall of the aorta forms the back wall of the RVOT.
Aortic translocation and biventricular outflow tract reconstruction. A new surgical repair for transposition of the great arteries associated with ventricular septal defect and pulmonary stenosis.
Pulmonary homografts and bovine jugular vein grafts can be used as conduits. These grafts can be rightward or leftward of the aorta, depending on the relation of the great arteries. If the aorta is transected, a Lecompte maneuver has also been described to reconstruct the RVOT.
Aortic translocation in the management of transposition of the great arteries with ventricular septal defect and pulmonary stenosis: results and follow-up.
In the “en bloc rotation techniques,” the native pulmonary valve can be used if size is adequate. If the pulmonary valve is hypoplastic, a monocusp patch is added to complete RVOT reconstruction. Growth potential is present as long as there is at least a posterior wall of native tissue.
An important question is whether these operations should preferentially be performed early in life, or whether they should be performed later. Many times cyanosis is only moderate and surgery can be postponed. If cyanosis warrants treatment, a modified Blalock shunt may be the first option, and many patients even have a second shunt so that reparative surgery can be performed at a later stage. Our preference is to have correction at a body weight of approximately 10 kg as we believe that a bigger RV to PA conduit will increase the interval to re-intervention. Other authors perform corrective surgery at a much younger age and prefer to avoid aortopulmonary shuts.
Effects of surgical en bloc rotation of the arterial trunk on the conduction system in children with transposition of the great arteries, ventricular septal defect and pulmonary stenosis.
The Rastelli operation remains a solution for patients with TGA, VSD, and LVOTO but has disadvantages. The 2 main issues are the LV to aortic tunnel and the extracardiac RV to PA conduit. Depending on the position of the aorta relative to the LV, the tunnel can be long and curvaceous. Growth is limited and fibrotic tissue can build up inside the tunnel. This may lead to subaortic stenosis that is difficult to repair adequately.
Fibrotic tissue can be enucleated from within the tunnel; however, when the anatomy of the tunnel is not changed, a high incidence of residual and recurrent stenosis remains. The LV to aortic tunnel reduces the volume of the RV.
Volume loss can be as high as 30% (Fig. 2). The defect in the anterior wall of the RV will increase RV dysfunction even further. As the RV to PA conduit is extracardiac, its durability is reduced compared with an orthotopically placed conduit.
As mentioned previously, when the LVOT or pulmonary valve is very hypoplastic, not much distance will be gained in a posterior direction, and the superiority of a Nikaidoh operation is here questionable.
Figure 2Left ventricular to aortic tunnel in the Rastelli operation. The right ventricular volume is reduced by the space that is occupied by the tunnel. VSD, ventricle septal defect; TV, tricuspid valve; position of VSD patch marked with the dotted line. (Color version of figure is available online.)
The Nikaidoh technique is clearly superior when the RV is small. In case of RV hypoplasia, a 1.5 ventricle repair may sometimes be obtained by combining a Nikaidoh procedure with a bidirectional Glenn shunt. When the aorta is distant from the LV, a Rastelli procedure will result in a long LV to aorta tunnel with 2 sharp angles, and here posterior aortic translocation will evidently provide for a much straighter connection between LV and aorta (Fig. 2).
When the VSD is remote or noncommitted, a Rastelli operation cannot be performed, whereas a Nikaidoh technique allows biventricular repair. A very small VSD makes a Rastelli procedure impossible, whereas posterior aortic translocation is still feasible. Even in the complete absence of an outlet VSD, biventricular repair can be performed using posterior translocation techniques.
Mitral or tricuspid valve straddling (through outlet or inlet VSD) sometimes impairs a Rastelli procedure, whereas a Nikaidoh technique may lead to biventricular repair. Severe mitral straddling can be a valid reason to refrain from biventricular repair altogether.
Differences between the REV operation and posterior translocation techniques are less pronounced (but not absent). An important part of the REV procedure is resection of the conal septum to make the connection between LV and aorta as direct as possible. Furthermore, the pulmonary trunk is directly (and usually nonvalved) implanted onto the RV to prevent a valved or a fully circular conduit between RV and PAs. A French (or Lecompte) maneuver is an essential part of the REV technique.
“En bloc rotation of the truncus arteriosus” will have the double advantage of placing the aortic valve in the LVOT and the pulmonary valve in the RVOT. Subpulmonary LVOT obstruction can usually be resected. In case of pulmonary valve hypoplasia, a Nikaidoh may be considered as a good alternative. When the pulmonary valve is adequately sized, an arterial switch operation with LVOT resection and VSD closure is the alternative to an “en bloc rotation” procedure.
The truncal half-turn maneuver or “en bloc rotation” techniques will have the same effect on the LV to aorta connection as the Nikaidoh procedure. Because of the rotation of the aorta, coronary arteries must be reimplanted.
Coronary artery anomalies greatly increase the risk of a Nikaidoh operation or in fact of any operation where the aorta has to be detached from the RV. When the RCA originates from the left anterior descending artery and crosses the RVOT from left to right, aortic detachment is best avoided. A circumflex artery from the RCA is not considered as a risk factor for the Nikaidoh technique but has been mentioned as a contraindication for the “en bloc truncal rotation techniques,” although with increasing experience, these coronary artery anomalies are not all seen as a contraindication.
The Bex-Nikaidoh procedure and in fact all its modifications, including “en bloc truncal rotation techniques,” have several drawbacks: coronary artery problems may occur due to transfer or due to leaving the coronary artery in place (kinking or stretching), aortic valve insufficiency has been reported to be a complication, and all over these operations are considered to be more technically demanding than the Rastelli procedure.
Aortic valve insufficiency has been reported as an important complication by several authors, and it must be assumed that a learning curve exists with these demanding procedures.
Results
There are many single-center studies that report the results of surgical treatment of TGA, VSD, and LVOTO. Most studies report on 1 technique.
En bloc rotation of the truncus arteriosus—an option for anatomic repair of transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction.
Effects of surgical en bloc rotation of the arterial trunk on the conduction system in children with transposition of the great arteries, ventricular septal defect and pulmonary stenosis.
Modified Nikaidoh procedure for the correction of complex forms of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction: mid-term results.
Anatomical factors determining surgical decision-making in patients with transposition of the great arteries with left ventricular outflow tract obstruction.
Surgery for transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction: European Congenital Heart Surgeons Association multicentre study.
All of the studies were retrospective, and none of the studies were randomized.
Reports on outcomes are listed in Table 1. Not all series in the literature have been included. We have focused on early and late mortality, on (freedom from) reinterventions, and on postoperative aortic insufficiency. Where possible, length of follow-up is mentioned. Leiden results with the Nikaidoh procedure have not been published previously.
Table 1Outcomes of Nikaidoh, Rastelli, REV, and En Bloc Rotation Operations
Aortic translocation in the management of transposition of the great arteries with ventricular septal defect and pulmonary stenosis: results and follow-up.
Modified Nikaidoh procedure for the correction of complex forms of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction: mid-term results.
Anatomical factors determining surgical decision-making in patients with transposition of the great arteries with left ventricular outflow tract obstruction.
Surgery for transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction: European Congenital Heart Surgeons Association multicentre study.
Surgery for transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction: European Congenital Heart Surgeons Association multicentre study.
In a study analyzing 25-year experience with the Rastelli operation in 101 patients with TGA, VSD, LVOTO, Kreutzer et al observed low early mortality but substantial late mortality and morbidity associated with RVOT or LVOT obstruction and arrhythmia. Freedom from death or reintervention was 53%, 24%, and 21% at 5, 10 and 15 years.
The European Congenital Heart Surgeons Association multicenter study including 82 patients also showed disappointing outcomes of the Rastelli operation with a high reoperation rate for RVOTO (10- and 15-year freedom from reoperation was 25% and 21%) and a not insignificant rate of reoperations for LVOTO (15-year freedom of 84%).
On the contrary, Brown et al, analyzing their 20-year experience with the Rastelli operation in 40 patients, reported better results: 20-year overall survival of 93%, freedom from RVOTO reoperations at 5, 10, 15, and 20 years of 86%, 74%, 63%, and 59%, and freedom from LVOTO reoperations at 20 years of 95%.
In the largest study analyzing long-term results of the REV procedure in 142 patients, Di Carlo et al reported overall survival of 85% at 25 years, 15-year freedom from RVOTO reoperations of 75%, and a very low incidence of LVOTO reoperations (2%).
In patients undergoing an REV procedure, the European Congenital Heart Surgeons Association multicenter study found freedom from death, reoperation, or catheter reintervention at 1, 5, and 10 years of 90%, 78%, and 64%, respectively. Freedom from reoperation for residual RVOTO and LVOTO at 10 years was 91% and 85%.
In a study reporting midterm results of the Nikaidoh procedure in 18 patients, Yeh et al reported 95% overall survival, 100% freedom from reoperations for LVOTO, 64% freedom from reoperations for RVOTO at 15 years, and no more than mild aortic regurgitation (AR).
Among the 17 patients undergoing the Nikaidoh procedure, Raju et al observed no mortality, no reoperation for LVOTO, and 24% reoperation rate for RVOTO. One patient required surgical valvuloplasty for moderate AR.
In a study of 14 patients who had undergone Nikaidoh procedure, Kramer et al noted 2 early and 1 late deaths, no reoperation for LVOTO, 2 reoperations for RVOTO, and no more than mild AR.
In our cohort of 13 patients with a Nikaidoh procedure (unpublished data), all survived, no patient needed reoperation for LVOTO, and only 1 patient had to undergo an RV-PA conduit change for endocarditis. All patients had mild AR or less.
In the series of 12 Nikaidoh patients, Morell et al noted 8.3% early and 0% late mortality. Two patients needed reoperation for RVOTO, and none of the patients was reoperated for LVOTO, 3 patients developed moderate AR.
In a study analyzing a 10-year experience with en bloc rotation technique in 13 patients, Mair et al reported no mortality, no reoperation for LVOTO, or RVOTO. One patient developed more than mild AR.
Discussion
Patients benefit most by an individualized surgical approach. There is not one single technique that fits all patients with this anomaly. Long-term follow-up shows that the Rastelli operation has several serious drawbacks related to an earlier need for reinterventions to the RVOT and a higher than desirable incidence of subaortic tunnel obstructions.
Surgery for transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction: European Congenital Heart Surgeons Association multicentre study.
In all operations where the aorta is posteriorly translocated, recurrent LVOTO has not been an issue, and it seems that reoperation for RVOTO or insufficiency occurs at the later stage than in patients who had a Rastelli procedure. It is particularly important, when the aorta is distant from the VSD, to avoid creation of a long and spiraling LV to aortic tunnel that can result in LVOTO and will also reduce RV volume. In this case, posterior aortic translocation is the best choice. Posterior aortic translocation is also advised when the VSD is noncommitted or very small, or when the RV is smaller than normal.
The REV operation and its modification by Metras also lead to a straight tunnel after resection of the conal septum but there have been some reports of reoperation for LVOTO.
Surgery for transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction: European Congenital Heart Surgeons Association multicentre study.
Coronary anomalies may complicate all procedures where the aorta is detached from the RV and here a Rastelli procedure may be still valid as alternative. When the pulmonary valve or LVOT is very small, not much is gained by posterior translocation of the aorta, so the Rastelli approach should be considered as a valid surgical alternative.
The aortic valve is at risk once the aorta is detached from the RV. Several strategies may be of help to minimize postoperative valve insufficiency. Partial aortic detachment is helpful in our experience, but others found that complete detachment aids in preventing AR.
Operating at an older age may reduce the risk for valve regurgitation: this is our policy and we feel that tissue handling is easier, and risk of aortic valve distortion is less in children who have a body weight of 8.0 kg or more. Aortic valve distortion is probably the most important disadvantage of Nikaidoh and truncal bloc rotation techniques, especially as residual or recurrent LVOTO is fully absent after these procedures, and RVOT reconstruction appears to be durable.
Comparisons between these operations are complicated because of different surgical eras and differences in follow-up and in numbers of included patients. Furthermore, there is probably a selection bias in selecting a specific technique and in surgical reporting.
References
Hazekamp M.G.
Portela F.
Bartelings M.
The optimal procedure for transposition of the great arteries and left ventricular outflow tract obstruction. An anatomical study.
Aortic translocation and biventricular outflow tract reconstruction. A new surgical repair for transposition of the great arteries associated with ventricular septal defect and pulmonary stenosis.
Aortic root translocation plus arterial switch for transposition of the great arteries with left ventricular outflow tract obstruction: intermediate-term results.
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).
En bloc rotation of the truncus arteriosus—an option for anatomic repair of transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction.
Aortic translocation in the management of transposition of the great arteries with ventricular septal defect and pulmonary stenosis: results and follow-up.
Effects of surgical en bloc rotation of the arterial trunk on the conduction system in children with transposition of the great arteries, ventricular septal defect and pulmonary stenosis.
Modified Nikaidoh procedure for the correction of complex forms of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction: mid-term results.
Anatomical factors determining surgical decision-making in patients with transposition of the great arteries with left ventricular outflow tract obstruction.
Surgery for transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction: European Congenital Heart Surgeons Association multicentre study.
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