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Ann Thorac Surg 2005;79:2089-2093
© 2005 The Society of Thoracic Surgeons

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Original article: Cardiovascular

Aortic Translocation in the Management of Transposition of the Great Arteries With Ventricular Septal Defect and Pulmonary Stenosis: Results and Follow-Up

^a Children’s Hospital of Pittsburgh, University of Pittsburgh, Pittsburgh, Pennsylvania
^b The Congenital Heart Institute of Florida, University of South Florida, Tampa, Florida

Accepted for publication November 17, 2004.

^_* Address reprint requests to Dr Morell, Division of Cardiothoracic Surgery, Children’s Hospital of Pittsburgh, University of Pittsburgh, Room 2820, 3705 Fifth Ave, Pittsburgh, PA 15213 (E-mail: victor.morell{at}chp.edu).

Presented at the Fiftieth Annual Meeting of the Southern Thoracic Surgical Association, Bonita Springs, FL, Nov 13–15, 2003.

	Abstract

Top Abstract Introduction Patients and Methods Results Comment Discussion Acknowledgments References

 
BACKGROUND: The surgical management of patients with transposition of the great arteries, ventricular septal defect, and pulmonary stenosis remains a challenge. The Rastelli operation is the preferred surgical procedure, but its long-term results are not optimal. The objective in this retrospective study was to review our experience using aortic translocation and biventricular outflow tract reconstruction as an alternative surgical procedure for the management of these patients.

METHODS: Since January 1996, 12 patients have undergone aortic translocation and biventricular outflow tract reconstruction for the management of transposition of the great arteries, ventricular septal defect, and pulmonary stenosis at our institution. All patients had ventriculoarterial discordance; 9 had atrioventricular concordance and 3 atrioventricular discordance. Associated lesions included a straddling atrioventricular valve in 3 patients. An inlet ventricular septal defect was present in 4 patients. The median age at operation was 2 years. Eight patients had previous palliative procedures. The surgical technique used was a modification of the Nikaidoh procedure. The 3 patients with atrioventricular discordance required a Senning procedure.

RESULTS: There was one hospital death (8.3%) as a result of a massive cerebrovascular accident. The median intensive care unit and hospital stays were 15 and 18 days, respectively. At a median follow-up of 33 months, all patients are alive. Four late reoperations occurred in 3 patients, including two reoperations for conduit obstruction.

CONCLUSIONS: Aortic translocation and biventricular outflow tract reconstruction is a valuable surgical option for the surgical management of patients with transposition of the great arteries, ventricular septal defect, and pulmonary stenosis, especially in the presence of "inadequate anatomy" for a Rastelli repair.

	Introduction

Top Abstract Introduction Patients and Methods Results Comment Discussion Acknowledgments References

 
Transposition of the great arteries (TGA) with a ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO) represents 0.67% of all congenital heart defects [1]. Although infrequent, this lesion remains a surgical challenge, commonly requiring palliation before proceeding with the definitive surgical repair.

In 1969 the Rastelli procedure was introduced for the surgical management of these patients [2, 3]. This repair represented a major advancement in congenital heart surgery because it allowed the preservation of the left ventricle as the systemic ventricle in patients with TGA, VSD, and LVOTO. The Rastelli repair has become the standard surgical technique for the management of this lesion, but its long-term results have been less than optimal. Common complications include conduit obstruction, LVOTO, and arrhythmias [4–8]. Maybe more importantly, the reported long-term survival has been surprisingly low [5, 6].

In 1984, Nikaidoh [9] introduced a new surgical approach for the management of TGA, VSD, and pulmonary stenosis (PS), which he called "aortic translocation and biventricular outflow tract reconstruction." This repair consists of harvesting the aortic root from the right ventricle (with attached coronary arteries), relieving the LVOTO (dividing the outlet septum and excising the pulmonary valve), reconstructing the left ventricular outflow tract (with the posteriorly translocated aortic root and the VSD patch), and reconstructing the right ventricular outflow tract (with a pericardial patch). Although technically challenging, this approach results in a more normal anatomic repair.

We are reporting our experience using a modified Nikaidoh procedure for the management of 12 patients with complex TGA with a VSD and PS.

	Patients and Methods

Top Abstract Introduction Patients and Methods Results Comment Discussion Acknowledgments References

 
Since January 1996, 16 patients underwent surgical repair of TGA, VSD, and PS at our institution. Twelve patients were managed with aortic translocation and biventricular outflow tract reconstruction, and 4 had a Rastelli type repair. The decision to proceed with either surgical technique was up to the operating surgeon. In the Rastelli group, 1 patient had a single coronary artery, which was thought to be a relative contraindication to aortic translocation because of the slightly increased surgical risk associated with the coronary transfer.

In the aortic translocation group, there were 10 boys and 2 girls, with a median age of 2 years (range, 5 months to 9 years). All patients had ventriculoarterial discordance, 9 had atrioventricular concordance, 3 had atrioventricular discordance (corrected transposition), and all had a VSD.

Ten patients had congenital valvar and subvalvar PS and 2 acquired valvar PS. The latter group consisted of 2 patients (1 atrioventricular concordance and 1 atrioventricular discordance) with straddling atrioventricular valves and no PS, who were initially managed with pulmonary artery banding to prevent pulmonary overcirculation. The pulmonary artery bands were placed too proximally in the main pulmonary artery, affecting the pulmonary valve development and precluding an arterial switch procedure.

Other associated cardiac anomalies included a straddling mitral (1 patient) and tricuspid valves (2 patients), cleft mitral valve (1 patient), multiple VSDs (1 patient), partial anomalous pulmonary venous return (1 patient), and the presence of the left anterior coronary artery crossing the right ventricular outflow tract (2 patients). An inlet VSD was present in 4 patients, and a restrictive VSD in 1. Eight patients had previous palliative surgeries, 6 systemic to pulmonary artery shunts and 2 pulmonary artery bands.

The surgical approach consisted of harvesting the aortic root from the right ventricle with individual coronary artery transfer, relieving the LVOTO by dividing the outlet septum and excising the pulmonary valve, performing the Lecompte maneuver, reconstructing the left ventricular outflow tract with the aortic autograft and the VSD patch, and reestablishing the right ventricular to pulmonary artery continuity using a pulmonary homograft. Also, in the 3 patients with atrioventricular discordance, an atrial switch operation (Senning) was performed.

	Results

Top Abstract Introduction Patients and Methods Results Comment Discussion Acknowledgments References

 
The median total bypass time was 260 minutes (range, 181 to 366 minutes), and the median cross-clamp time was 185 minutes (range, 149 to 258 minutes). The median length of stay in the intensive care unit was 15 days (range, 5 days to 3 months), with a median hospital stay of 18 days (range, 6 days to 3 months). There was 1 hospital death (8.3%) as a result of a massive cerebrovascular accident.

Three patients required early reoperations: 1 for postoperative bleeding, 1 for a residual VSD, and 1 for a mitral valve repair. One patient required a pacemaker placement for complete heart block. Four late reoperations occurred in 3 patients, 2 for conduit replacements (at 1.5 years and 2.5 years after the initial procedure), 1 for mitral and tricuspid valve repair, and 1 for an abnormal granulomatous reaction to a mediastinal polytetrafluoroethylene membrane requiring mediastinal exploration.

At a median follow-up of 33 months (range, 21 to 57 months), all patients are alive. The ventricular function is normal in 10 patients and mildly decreased in 1. Three patients experienced moderate aortic insufficiency. Late atrial arrhythmias were noted in 4 patients (ectopic atrial tachycardia [n = 1], sick sinus syndrome [n = 1], junctional rhythm [n = 1], and atrial flutter [n = 1]). Nine of the survivors are in New York Heart Association class I and 2 are in class II. In the latter group, both patients have residual hemodynamic lesions consisting of a large apical VSD in one and moderate aortic insufficiency in the other.

	Comment

Top Abstract Introduction Patients and Methods Results Comment Discussion Acknowledgments References

 
The surgical reestablishment of normal cardiac anatomy is a major goal in congenital heart surgery. We have witnessed an evolution in the management of TGA, in which the highly successful atrial switch procedure has been replaced by the arterial switch procedure, which results in a more acceptable anatomic repair. It has been surmised that this will result in improved long-term outcome.

Since its introduction in 1969, the Rastelli repair has become the "gold standard" in the surgical management of patients with TGA, VSD, and PS. This repair has proven to be very useful in the management of these patients, with an acceptable early mortality, but its long-term results are not optimal. Conduit obstruction is frequently reported and appears to be related to its nonanatomic location in the right ventricular outflow tract, which makes it prone to sternal compression. Left ventricular outflow tract obstruction at the level of the VSD or at the level of the intraventricular tunnel occurs in up to 10% of patients [5].

The Boston Children’s Hospital reported their experience with the Rastelli repair in 101 patients with atrioventricular concordance and a VSD (LVOTO was present in 91 patients) during a 25-year period [5]. The overall mortality was 7%. Freedom from reintervention for right-sided obstruction was 21% at 15 years, and there was a 10% incidence of LVOTO. The overall freedom from death or transplantation at 20 years was 52%. Surprisingly, the Mayo Clinic (Rochester, MN) published similar results with a 59% survival at 20 years [6]. The reported long-term results from these two respectable institutions raise questions about the long-term physiologic implications of the Rastelli repair. Graham and associates [10] have reported abnormal contractile function and increased mean end-systolic stress and left ventricular mass in patients after a Rastelli repair.

There are several anatomic variables that complicate the performance of a Rastelli repair. The abnormal course of a major coronary artery crossing the right ventricular outflow tract could prevent the performance of a distal right ventriculotomy. The presence of an inlet or restrictive VSD or a straddling mitral or tricuspid valve may prove to be major anatomic obstacles in creating the intraventricular tunnel. Interestingly, the presence of a straddling tricuspid valve has been associated with increased early and late mortality after a Rastelli repair [5]. Also, the right ventricular volume is partially compromised by the interventricular tunnel; thus, the presence of right ventricular hypoplasia can be considered a contraindication to a Rastelli repair.

Certainly, in patients with inadequate anatomy for a Rastelli repair, aortic translocation becomes an attractive alternative. Although technically challenging, aortic translocation combines elements of commonly performed surgical techniques including the Ross, Konno, and Jatene procedures. In the original technique described by Nikaidoh [9], the aortic root was mobilized and translocated without detaching the coronary arteries. Coronary insufficiency has been a concern with this technique and that is why we opted to proceed with individual coronary artery transfer during translocation. During aortic translocation, the aortic root is moved posteriorly a distance equal to the sum of the thickness of the outlet septum and the diameter of the pulmonary valve. The greater the distance involved in the actual translocation of the aortic root, the greater the likelihood for the development of coronary insufficiency. Another technical concern is the development of right pulmonary artery stenosis by the posterior displacement of the aortic root. The Lecompte maneuver could help prevent this possible complication. Del Nido and associates [11] reported excellent results in 4 patients with TGA, VSD, and LVOTO using a surgical approach similar to ours.

In the experience of Nikaidoh and Leonard [12], 3 of 14 patients required postoperative extracorporeal membrane oxygenation support (1 patient died), and we wonder whether coronary insufficiency played a role. Their results are certainly impressive with 1 early death and no late mortality with a follow-up period of more than 17 years. Four patients (30%) required late reoperations.

When using this technique, we have noticed that once the aorta has been harvested from the right ventricle and the outlet septum divided, the exposure provides for an excellent visualization of the VSD and the atrioventricular valve. Thus, the closure of the VSD is easier and more precise in patients who have a straddling atrioventricular valve.

Unusual coronary anatomy has been found to be a risk factor for increased mortality in patients undergoing an arterial switch operation [13–15]. In these patients, the Rastelli procedure avoids the risks related to coronary transfer and preserves the left ventricle as the systemic ventricle. Therefore, difficult coronary anatomy could be considered a relative contraindication to aortic translocation. Certainly, as we gain more experience with challenging coronary artery transfers, some of these patients could be considered for aortic translocation.

Aortic translocation and biventricular outflow tract obstruction results in a more normal anatomic repair (Fig 1), which could result in improved cardiac performance and long-term survival. The right and left ventricular outflow tracts are better aligned, avoiding the right angle turns created by the Rastelli repair. The right ventricle to pulmonary artery conduit originates normally from the right ventricle and is therefore less susceptible to sternal compression. This may result in improved conduit longevity with a decreased incidence of reoperation.

View larger version (36K): [in this window] [in a new window]   Fig 1. (A) Lateral view of the heart with transposition of the great arteries, ventricular septal defect (VSD), and pulmonary stenosis. (B) Lateral view of the same heart with a classic Rastelli procedure. Although a tried and true procedure, this demonstrates that the outflow of each ventricle must make a right angle turn, pass through a muscular tunnel (either a ventricular septal defect or a ventriculotomy), and continue in an anterior conduit subject to compression by the sternum. (C) Lateral view of the same heart with the aortic valve transferred posteriorly to the left ventricular outflow tract and the pulmonary artery reconstruction accomplished directly end to end to the right ventricular outflow tract. Ventricular outflow is straight and direct, and unobstructed without risk of sternal compression by the sternum. (Ao = aorta; LA = left atrium; LV = left ventricle; PA = pulmonary artery; RV = right ventricle.) (Reprinted from Haas GS, Curr Opin Pediatr; 2000;12:501–4[16], with permission.)

We report patients with corrected TGA, VSD, and PS who have undergone a "modified double switch" procedure consisting of aortic translocation and biventricular outflow tract obstruction with an atrial switch procedure. In this lesion the conduction tissue runs anterior and cephalad to the pulmonary valve and then descends along the anterior margin of the VSD before diverging into the bundle branches [17]. Division of the outlet septum should not result in complete heart block, but care should be taken when suturing the VSD patch not to injure the conduction tissue. One of 3 patients experienced complete heart block in our series.

Aortic translocation and biventricular outflow tract reconstruction deserve consideration in the surgical management of patients with TGA, VSD, and PS, especially in the presence of an inlet or restrictive VSD, a straddling atrioventricular valve, a small right ventricle, and an abnormal coronary artery pattern. This surgical repair results in a more anatomically correct alignment between the ventricles and their respective great arteries, and this could result in better long-term functional outcome. Certainly, a larger number of patients and longer follow-up are required to fully assess the potential long-term benefits of this procedure compared with the Rastelli repair.

	Discussion

Top Abstract Introduction Patients and Methods Results Comment Discussion Acknowledgments References

 
DR ROSS M. UNGERLEIDER (Portland, OR): Victor, you are to be congratulated on taking a very innovative technique and finding a way to make it more innovative and possibly even more reproducible. It is a wonderful series.

The question that I have for you is to explain to us how doing a Lecompte maneuver places the right ventricle to pulmonary artery conduit in a more posterior location. I think that it is clear from the description of this operation that the advantage would be a reduced potential for left ventricular outflow tract obstruction, but I do worry about the conduit in this location in this group of patients being more prone to sternal compression, more prone to long-term pulmonary insufficiency, and I am not sure that you have really solved that problem, and, as you say, more follow-up will be required.

But it is a magnificent operation, it is imaginative, and it is exactly the kind of thing that we as congenital heart surgeons should continue, suggesting to try to further improve what we do and create progress and change. Thank you.

DR MORELL: Thank you Ross. During aortic translocation, the aorta is moved posteriorly and the homograft used to reconstruct the right ventricular outflow tract is placed anterior to the aortic root, essentially originating from the aortic harvest site in the right ventricle. In a Rastelli repair, the homograph originates from the right ventricular outflow tract in a very nonanatomic location, making it more prone to sternal compression. The homograph placed during aortic translocation certainly sits in a more posterior location in the mediastinum.

DR UNGERLEIDER: And again I would just point out that even when doing a classic arterial switch and a Lecompte maneuver, we are still moving the aorta posteriorly, and I am impressed with how anterior those right ventricular outflow tracts are, but you have obviously seen this and it looks different. It would be interesting to see when you publish the manuscript perhaps side by side lateral angiograms of the conduit and the location that you place in these patients compared to those that get a normal Rastelli.

DR ANDREW C. FIORE (St. Louis, MO): Did you have to enlarge the ventricular septal defect in any of those patients?

DR MORELL: During aortic translocation, the outlet septum is divided, which essentially enlarges the ventricular septal defect and the left ventricular outflow tract.

	Acknowledgments

Top Abstract Introduction Patients and Methods Results Comment Discussion Acknowledgments References

 
We would like to acknowledge our late partner Gary Haas, MD, for his significant clinical contribution to this series.

	References

Top Abstract Introduction Patients and Methods Results Comment Discussion Acknowledgments References

 

1. Keith JD, Rowe RD, Vlad P. Heart disease in infancy and childhood. New York: MacMillan; 1978.
2. Rastelli GC. A new approach to the "anatomic" repair of transposition of the great arteries Mayo Clin Proc 1969;44:1-12.[Medline]
3. Rastelli GC, Wallace RB, Ongley PA. Complete repair of transposition of the great arteries with pulmonary stenosisa review and report of a case corrected by a new surgical technique. Circulation 1969;39:83-95.[Abstract/Free Full Text]
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12. Nikaidoh H, Leonard SR. Aortic translocation and biventricular outflow tract reconstruction for d-transposition associated with ventricular septal defect and pulmonary stenosis. a follow-up. 2001Presented at the Third World Congress of Pediatric Cardiology and Cardiac Surgery.
13. Mayer JE, Sanders SP, Jonas RA, et al. Coronary artery pattern and outcome of arterial switch operation for transposition of the great arteries Circulation 1990;82(Suppl 5):IV-139-IV-145.
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