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Ann Thorac Surg 2002;73:88-95
© 2002 The Society of Thoracic Surgeons


Original article: cardiovascular

Successful late reintervention after the arterial switch procedure

Sanjiv K. Gandhi, MD*a, Frank A. Pigula, MDa, Ralph D. Siewers, MDa

a Division of Pediatric Cardiothoracic Surgery, Department of Surgery, Children’s Hospital of Pittsburgh, University of Pittsburgh, Pittsburgh, Pennsylvania, USA

* Address reprint requests to Dr Gandhi, Division of Pediatric Cardiothoracic Surgery, Children’s Hospital of Pittsburgh, Suite 2820, 3705 Fifth Ave, Pittsburgh, PA 15213-2583, USA
e-mail: gandhis{at}heart.chp.edu

Presented at the Thirty-seventh Annual Meeting of The Society of Thoracic Surgeons, New Orleans, LA, Jan 29–31, 2001.


    Abstract
 Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 Acknowledgments
 Discussion
 References
 
Background. The arterial switch operation is the therapy of choice for transposition of the great arteries.

Methods. A retrospective analysis of all children undergoing the arterial swtich operation between November 1985 and October 2000 was conducted, highlighting the frequency and nature of late invasive reintervention.

Results. One hundred forty-four children were operated on. Operative survival was 89% (128 of 144). Late reintervention was required in 23% (29 of 128) of survivors. Neopulmonary stenosis (PS) was the most common complication requiring treatment, occurring in 16% (21 of 128) of patients. Eleven of 21 patients with PS required reoperation, whereas 10 were managed with percutaneous techniques. Other indications for reintervention included aortic arch obstruction (3 patients), ventricular septal defect (with PS in 2 patients), bronchial stenosis (2 patients), coronary stenosis (2 patients), aortic stenosis (with PS in 1 patient), and residual atrial septal defect (1 patient). There has been no mortality or major morbidity in those children who have undergone reintervention.

Conclusions. Invasive reintervention after the arterial switch operation is occasionally required. The most common indication is PS. Reintervention is well tolerated by those children who require it. Continued follow-up for late complications is required in this patient population.


    Introduction
 Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 Acknowledgments
 Discussion
 References
 
The arterial switch operation (ASO) is widely recognized as the therapy of choice for children born with transposition of the great arteries. As experience with this operation has increased, mortality probability has been lowered and is consistently below 10% in most contemporary series [15]. Most children who have undergone the ASO have also enjoyed normal growth, development, and cardiac function. In contrast to the former atrial switch procedures, the ASO has the advantages of maintenance of sinus node function, preservation of the left ventricle as the systemic ventricle, and the mitral valve as the systemic atrioventricular valve. However, the ASO involves translocation of the coronary arteries, the pulmonary valve becomes the systemic outflow valve, and the pulmonary arteries may become distorted because of their atypical relationship to the great vessels. Late reintervention, whether in the form of therapeutic cardiac catheterization or operation, has not been uncommon in this patient population [610]. It is the purpose of this study to define the frequency and nature of the complications that have required late invasive reintervention in a series of more than 140 patients operated on more than 15 years at a single institution.


    Material and methods
 Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 Acknowledgments
 Discussion
 References
 
Patients
A retrospective analysis of all children undergoing the ASO between November 1985 and October 2000 at the Children’s Hospital of Pittsburgh was conducted, highlighting the frequency and nature of late invasive reintervention. Late reintervention was defined as an intervention performed after hospital discharge and beyond the first 30 postoperative days; such procedures were generally performed electively.

Follow-up
All patients were evaluated in the surgery office 1 week postoperatively. Subsequently, they were examined by their cardiologist at 3-month intervals during the first year, 6-month intervals during the second year, and then yearly. This protocol was modified if there were any specific problems identified that required closer follow-up. Patients received postoperative echocardiograms at the discretion of the treating cardiologist, based on symptoms and physical examination. The first 60 patients in the series received routine cardiac catheterization within the first postoperative year. There were no asymptomatic coronary problems detected, therefore, for subsequent patients, decisions to perform cardiac catheterization were based on: (1) echocardiographic findings of abnormal function or mitral regurgitation; (2) right ventricular/left ventricular (RV/LV) pressure ratio greater than 0.5; (3) progressively increasing right ventricular outflow tract gradient over time; or (4) right ventricular outflow tract gradient more than 30 mm Hg. Oftentimes, before angiography, a differential V/Q pulmonary scan was also performed to delineate differential pulmonary blood flow when the echocardiogram demonstrated increased flow velocity at the bifurcation or in the supravalvular main pulmonary artery.

Statistical analysis
All data values are expressed as mean ± standard deviation. Statistical significance of all paired data was determined by Student’s paired t test.


    Results
 Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 Acknowledgments
 Discussion
 References
 
Between November 1985 and October 2000, 144 children with various forms of transposition of the great arteries were operated on in our institution. Sixty-nine percent had an intact ventricular septum and 31% had an associated ventricular septal defect. Mean age at operation was 13 ± 15 days. Neonatal repair was performed in 94% (135 of 144) of patients.

One hundred forty-four children were operated on. Overall operative survival was 89% (128 of 144), but has declined significantly as the experience has increased (83% [63 of 76] during the first 7 years of the experience and 96% [65 of 68] over the latter 7 years [p = 0.03]). Follow-up has been 100% complete. Mean follow-up is 74 ± 45 months. Late reintervention was required in 23% (29 of 128) of survivors (Table 1). Postoperative complications severe enough to require late invasive reintervention can be divided into neopulmonary stenosis (PS) and all others (Table 2).


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Table 1. Patients Requiring Late Reintervention After Arterial Switch Operation

 

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Table 2. Indications for Late Reintervention After Arterial Switch Operation

 
Neopulmonary stenosis
Neopulmonary stenosis was the most common complication requiring treatment, occurring in 16% (21 of 128) of surviving patients (Fig 1). Eleven of 21 patients have required reoperation, whereas 10 were managed with percutaneous techniques. One patient underwent surgery 5 years after pulmonary artery stent placement for recurrent stenosis (Fig 2). The mean interval from the time of ASO to the time of reintervention for PS was 44 ± 30 months. Only 8% (5 of 65) of survivors in the latter half of the experience have required reintervention for neopulmonary stenosis and, of these, only 2 patients (3%) have thus far required surgery.



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Fig 1. Pulmonary angiogram in a patient with neopulmonary stenosis involving main and branch pulmonary arteries, 91 months after arterial switch operation.

 


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Fig 2. Recurrent neopulmonary stenosis. This patient had a left pulmonary artery stent placed 10 years after the arterial switch operation and had surgical repair of the recurrent stenosis 5 years later.

 
The location of PS varied (Table 3). Fifteen percent (3 of 21) of patients had mainly infundibular combined with valvular stenosis. Ten percent (2 of 21) of children were found predominantly to have a combination of valvular and supravalvular stenosis. The majority of patients (76%, 16 of 21) suffered supravalvular stenosis; of these patients, 69% (11 of 16) had main pulmonary artery or main pulmonary artery along with branch pulmonary artery stenosis, whereas 31% (5 of 16) had isolated left pulmonary artery stenosis. The operative findings of each of the 5 patients with valve involvement were carefully reviewed; a frankly dysplastic valve was noted in only one case, and thickened valves with relatively small annuli were discovered in the remainder.


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Table 3. Location of Neopulmonary Stenosis

 
Several risk factors for the occurrence of postoperative PS were examined. The presence of coronary anomalies was not statistically significant (p = 0.6) (Table 4). There was no difference in the incidence of PS based on the presence of a ventricular septal defect (p = 0.5) (Table 5). Finally, over the 15-year time period of this study, three different materials were employed for pulmonary sinus reconstruction, including bovine pericardium, cryopreserved pulmonary homograft, and autologous fresh pericardium. Although the use of autologous pericardium had a low incidence of PS, it was used infrequently. There was no measurable difference with respect to the occurrence of postoperative PS based on the type of material used to augment the neopulmonary artery (p = 0.7) (Table 6).


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Table 4. Coronary Pattern and Relationship With Neopulmonary Stenosis

 

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Table 5. Relationship of Ventricular Septal Defects With Neopulmonary Stenosis

 

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Table 6. Relationship of Materials Used to Reconstruct Pulmonary Sinus With Neopulmonary Stenosis

 
Other complications
Other indications for reintervention included aortic arch obstruction (3 patients), ventricular septal defect (with PS in 2 patients), bronchial stenosis (2 patients) (Fig 3), coronary stenosis (2 patients) (Fig 4), aortic stenosis (with PS in 1 patient), and residual atrial septal defect in 1 patient. The 2 patients with bronchial compression were treated with modified aortopexy. The patients with coronary stenosis were treated with bypass grafting. Two of the 3 patients with arch obstruction have had balloon angioplasty (Fig 5), and the other patient underwent surgical repair. There has been no mortality or major morbidity in those children who have undergone reintervention.



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Fig 3. Magnetic resonance image illustrating compression of the left mainstem bronchus by the neoaorta discovered 19 months after the arterial switch operation.

 


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Fig 4. Obstruction of the left coronary artery 12 months after an arterial switch procedure. The right coronary artery is seen to opacify well but the left system fills only through retrograde collaterals.

 


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Fig 5. (A) Aortic arch obstruction after the arterial switch managed with invasive cardiac catheterization techniques. (B) Aortic arch seen in Panel A after balloon angioplasty of obstruction.

 

    Comment
 Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 Acknowledgments
 Discussion
 References
 
Mortality after the ASO has been consistently lowered to less than 10%. However, reoperative probability for a variety of complications has ranged from 5% to 30% in the literature [110]. Complications significant enough to require invasive reintervention can be classified into right ventricular outflow tract lesions and all others.

Neopulmonary stenosis
Neopulmonary stenosis may occur in the subvalvular [11, 12], valvular [13], or supravalvular regions. Several theories have been offered to explain the significant occurrence of this postoperative complication after the ASO. The neopulmonary valve annulus may be abnormally small after the ASO [14]. The tendency for the neopulmonary valve diameter to be smaller than normal after the ASO is in contrast to overgrowth of the neoaortic valve after the switch operation. The neopulmonary annulus may exhibit a tendency to be compressed by the dilated neoaorta and may not be circular. Morphologic analyses of specimens of complete transposition have revealed variations that produce a sharper angulation between ventricular inflow and outflow when compared with the normal situation, accentuated in those specimens with a ventricular septal defect [11, 12]. Discrete outflow obstruction has been discovered due to deviation of the outlet septum, anomalous muscle bundles, hypertrophy of septomarginal and septoparietal trabeculations, and circumferential infundibular hypertrophy. Other postulations for neopulmonary stenosis include inadequate growth of the main pulmonary artery resulting in flattening of the reconstructed right ventricular outflow tract, circumferential narrowing of the suture line, and ductal tissue at the origin of the left pulmonary artery causing a coarctation of the left pulmonary artery [15]. Some authors have suggested that postoperative modification of the neosubpulmonary flow may be the source of turbulences and right ventricular outflow tract obstruction [12].

Whether a specific material utilized to perform pulmonary sinus reconstruction accelerates the development of PS is controversial. It has been suggested that materials other than autologous fresh pericardium perpetuate a higher incidence of late obstruction [6, 1618]. Some believe the more viable the pulmonary patch, the lower the rate of late pulmonary stenosis [10]. Sakurai and associates [16] compared the use of glutaraldehyde-treated heterologous pericardium and fresh autologous pericardium in 50 consecutive survivors of the ASO. Nine of 35 patients in the former group but none in the latter group required reintervention for PS. Carrel and associates [17] reported excellent hemodynamic results and a very low incidence of reoperation for PS using direct reconstruction of the neopulmonary artery. Although our study was not randomized, it did not implicate the use of any specific material in the occurrence of postoperative PS.

Neopulmonary stenosis in our series was predominantly observed at the supravalvular level. It is difficult to implicate morphologic explanations as being substrates for supravalvular PS. Preoperative anatomic differences were also not statistically significant factors. Although our series is not large enough to establish statistical significance, we believe there is a declining incidence of PS that is consequent to a gradual increased appreciation of the need to avoid tension on the anastomosis of the neopulmonary artery. More extensive mobilization of the distal pulmonary arteries and more generous pantaloon patches may have helped to ameliorate the occurrence of postoperative PS. The fact that the location of PS varies between institutions reinforces the hypothesis that technical factors probably contribute significantly to this problem.

Williams and associates [6], in a multiinstitutional study, reported the risk-unadjusted freedom from reintervention for right-sided obstruction to be 94%, 88%, and 83% at 1, 5, and 10 years, respectively. The instantaneous risk of such reintervention rose to a peak at 9 months. Others have also emphasized the early occurrence of postoperative pulmonary stenosis within the first year with progressive accentuation. In our population, the mean interval from the time of ASO to the time of reintervention for PS was 3 to 4 years, suggesting that this problem is not restricted to the first postoperative year and that longer follow-up is mandated to truly appreciate the magnitude of the problem [7].

The benefit of percutaneous intervention in those children in whom it was performed has been variable. Balloon angioplasty is useful when the stenosis involves the branch pulmonary arteries or is remote from the valve. Stenosis location involving the main pulmonary artery or pulmonary bifurcation is best treated by reoperation. Stent placement does significantly complicate reoperation, and stent durability in this disease is unproven.

Other complications
Complications other than neopulmonary stenosis after the arterial switch operation severe enough to require reintervention are distinctly uncommon [19]. As previously elucidated [20, 21], the mechanism of left bronchial compression after an arterial switch operation may relate to the placement of the proximal ascending aorta posterior to the transected pulmonary artery. The left main bronchus is intimately related to the left pulmonary artery and the descending thoracic aorta as it courses beneath the aortic arch. The mobilization and posterior displacement of the ascending aorta behind the left pulmonary artery as the neoaorta may allow it to impinge upon the left main bronchus, or may permit compression of the bronchus between the ascending and descending aorta.

Aortic arch obstruction seems to be limited to that patient population in whom an aortic coarctation was present and an extensive aortic reconstruction comprised a portion of the initial operation. This problem appears, at least in the short term, amenable to balloon angioplasty techniques for correction.

Others have reported varying rates of aortic insufficiency after the ASO [4, 22]. It has been postulated that the regions of aortic and coronary sutures are a site of turbulences that may cause aortic root enlargement in its weakest portions. We did not observe this complication severe enough to require reintervention.

Limitations of the study
This series comprises 144 patients operated on by 4 different surgeons for more than 15 years. Although the essential surgical technique and philosophy between individual surgeons were similar, subtle variations and changes over the time period of the study may have influenced the development of certain postoperative complications. Reintervention was utilized as the criteria for complications. Whereas this is a finite unarguable end point and the focus of study, it may have altered the actual incidence of complications.

Conclusions
Invasive reintervention after the ASO is occasionally required. The most common indication is PS. The reasons for PS are multifactorial. Reintervention is well tolerated by those children who require it. Continued follow-up for late complications is required in this patient population.


    Acknowledgments
 Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 Acknowledgments
 Discussion
 References
 
We thank the Division of Cardiology at the Children’s Hospital of Pittsburgh for their invaluable assistance in the preoperative care and postoperative follow-up of these children. We would also like to recognize Drs Pedro del Nido and John Meyers for their contributions as operating surgeons on several of the patients in this series.


    Discussion
 Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 Acknowledgments
 Discussion
 References
 
DR KIRK R. KANTER (Atlanta, GA): Doctor Gandhi, very nice presentation and nice series. I have two questions for you.

Is the development of pulmonary stenosis time related? Is it a continuous variable in that it increases linearly with time, or is there a risk period where it is more common and then the risk goes down?

And secondly, I think there is a paper from the Congenital Heart Surgeons’ Society that showed that the use of a pantaloon patch seemed to markedly reduce the risk of late pulmonary stenosis regardless of the material used to reconstruct it. Can you comment on the operative technique of how the harvest sites at the old ascending aorta were patched and what technique was used?

DR GANDHI: Thank you Dr Kanter. With respect to the first question, I did not present the data, but the development of neopulmonary stenosis is, in fact, time related. It occurred a mean of between 3 and 4 years after the original operation for transposition. Kaplan-Meier analysis demonstrates that the risk continues for the length of follow-up, in concordance with the Congenital Heart Surgery Society’s (CHSS) data.

The CHSS actually separated the patients into those with proximal and distal right ventricular outflow tract obstruction. The hazard function for proximal obstruction consisted of a single peaking hazard phase, in contrast to the hazard function for distal obstruction that consisted of both an early peaking hazard phase and a constant hazard phase that persisted throughout the length of follow up.

The CHSS data also demonstrated that there was a significant difference in the incidence of neopulmonary stenosis with the use of nontreated autologous pericardium in reconstruction of the pulmonary sinus. We did not find that to be the case. For the first few patients in the series, bovine pericardium was utilized to reconstruct the pulmonary sinus. Autologous pericardium and cryopreserved pulmonary homograft have been employed for the remainder of the patients. We use a pantaloon patch and excise generous buttons in the standard fashion. These factors have not seemed to make a difference, at least in our series, accepting the fact that this is not necessarily the case in the CHSS data or in other isolated series in the literature.

DR CHARLES D. FRASER, JR (Houston, TX): Thank you for that really provocative presentation, and it does bring to mind the issues that we have struggled with.

But I wanted to ask you, firstly, you stated that the coronary artery branching pattern did not seem to have an impact on the incidence of problematic pulmonic stenosis. I assume also coronary restenosis. But I was wondering if you included Taussig-Bing patients in this series?

In our own series, although not a predictor of mortality, the patients with Taussig-Bing anomaly, because of the relationship of the great vessels, have a bit higher incidence of pulmonary branch problems.

And then the other issue that we have to grapple with is how do you follow patients after arterial switches? Should they have annual coronary angiography, or is echo adequate for that? I’m interested in your thoughts.

DR GANDHI: Thanks for your comments. As far as the coronary patterns go, they were not significant in our series. But again, in the CHSS series, which represents a much larger group of patients, Dr Williams and associates found that there was a significantly increased incidence of pulmonary stenosis when the left coronary artery originated from sinus 2.

We did include Taussig-Bings in our series. I think that 9 of the total 144 patients were Taussig-Bings. I cannot honestly tell you if there was a relationship between that particular anatomy and the presence of neopulmonary stenosis.

Your comments regarding follow-up are extremely important. The first 60 patients in our series, until mid 1991, had routine cardiac caths in the first postoperative year. No asymptomatic coronary lesions were identified among those 60 patients. There were 2 total patients in our series who had coronary problems discovered. One was an infant who presented in heart failure, as I discussed, and the other was an infant who presented with mitral regurgitation found on echocardiogram, which was followed up by a cardiac cath that revealed a left coronary problem. We no longer perform routine postoperative cardiac caths. The kids are followed with serial echoes. We think that this is an appropriate way to follow these children, as routine cardiac caths did not seem to be beneficial in discovering unsuspected problems.

With respect to neopulmonary stenosis, one of the other things we often do if neopulmonary stenosis is found on echocardiography is a differential V/Q scan to quantitate the distribution of pulmonary blood flow.

Dr JOHN W. BROWN (Indianapolis, IN): I enjoyed your presentation, and I think you bring to light a concern that all of us have. The incidence of pulmonary stenosis after arterial switch just seems too high. We reviewed and presented our results at this meeting last year, and our overall incidence of pulmonary stenosis for a series of 200 patients is less than 4%, followed carefully by our cardiologists.

I cannot help but think there is one of two mechanisms causing your higher than expected incidence of pulmonary stenosis. Either you are not mobilizing the branch pulmonary arteries enough and there is tension on the pulmonary anastomoses; or the type of pantaloon patch that you are putting in the neopulmonary artery is not quite big enough. We stress that making a real big, bug-eyed patch in the neopulmonary sinuses is important. When you look at the pulmonary root, it has two big bulging patches where the coronaries came from. And I cannot help but think that just minor modifications in your technique will significantly reduce your pulmonary stenosis problem.

DR GANDHI: I appreciate your comments. The incidence of pulmonary stenosis in this series is really the incidence of reintervention for pulmonary stenosis. Intuitively, you may think that we have underestimated our incidence of pulmonary stenosis because we have not reported those children with 10 or 15-mm Hg gradients who have not undergone balloon angioplasty, stenting, or reoperation. However, we may actually be "overestimating" the incidence of truly significant pulmonary stenosis; when you talk to our cardiologists, often a lesion that appears angiographically amenable to angioplasty is balloon dilated; this is consequently manifested in our database as a reintervention despite the fact that the gradient may have only been 15 or 20 or 25 mm Hg. Having said this, I do appreciate your concerns. We do mobilize the pulmonary arteries all the way out to the hilum and have been more generous, I think, in recent years with the size of our pantaloon patch. Hopefully, we will start to reap the benefits of these technical modifications.

DR ROBERTO M. DiDONATO (Rome, Italy): One problem we are observing in our experience that you do not seem to see in your series is that of aortic insufficiency (ie, neoaortic insufficiency). We see that in a fair number of cases, and I apologize for not giving you the exact figure.

We speculate that this might be due to the way we reimplant the coronary arteries. We actually perform coronary reimplantation in a standard fashion, carrying out the anastomoses into vertical incisions of the free edge of the neoaortic root. We also use the trapdoor technique. We think that this method may lead to geometrical distortion of the sinotubular junction leading to malalignment of the commissures and secondary aortic insufficiency.

My question is if you have looked into this issue and if you have any consideration to do about it?

DR GANDHI: Although other series have reported aortic insufficiency, we have not observed aortic insufficiency significant enough to reintervene. Again, I would like to reemphasize the point that this is a series in which complications are based upon reintervention, whether that reintervention is an operation or an invasive catheterization procedure. Reintervention is the finite, unarguable end point we used to identify complications. So, if there are children in our series who have mild or mild to moderate aortic insufficiency that the cardiologists are simply observing, they have yet to be classified as a "late complication" of the arterial switch procedure.


    References
 Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 Acknowledgments
 Discussion
 References
 

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