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Ann Thorac Surg 1997;64:1776-1781
© 1997 The Society of Thoracic Surgeons
Divisions of Cardiovascular Surgery and Cardiology, The Montreal Children's Hospital, McGill University, Montreal, Canada
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Abstract |
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 TopFootnotes Abstract IntroductionPatients and MethodsResultsCommentAddendumReferences |
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Methods. Since 1989, 12 of 13 patients with aortic arch obstruction and transposition of the great arteries or double-outlet right ventricle with subpulmonary ventricular septal defect have undergone complete single-stage repair. One patient underwent a two-stage repair because of hemodynamic instability. The median age of repair was 27 days and the median weight was 3.5 kg. Surgical technique involved the arterial switch operation and ventricular septal defect closure when present in 12 patients. One patient with severe subaortic stenosis underwent a modified Damus-Kaye-Stansel operation with concomitant aortic arch enlargement. The aortic arch was enlarged in 12 of 13 patients with a pulmonary homograft patch.
Results. There have been no early deaths and only one late death at 39 months postoperatively from hepatoblastoma. The mean follow-up is 42 months. There have been no reoperations for recurrent aortic arch obstruction. All survivors are currently well from a cardiac point of view.
Conclusions. Concomitant single-stage repair for transposition complexes with aortic arch obstruction achieves excellent survival and should be the surgical procedure of choice.
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Introduction |
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TopFootnotesAbstractIntroductionPatients and MethodsResultsCommentAddendumReferences |
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The arterial switch operation (ASO) has become the most common repair for transposition of the great arteries (TGA) with intact ventricular septum or ventricular septal defect (VSD) [1]. Some also consider it the procedure of choice for double outlet right ventricle with a subpulmonary VSD (the Taussig-Bing heart) [2, 3]. However, in association with aortic arch obstruction (AAO), the traditional approach has been to repair the coarctation and hypoplastic aortic arch through a left thoracotomy and concurrently palliate the cardiac malformation with a pulmonary artery band (PAB) [4–6]. At a later stage, patients undergo intracardiac repair and debanding through a median sternotomy. This two-stage approach has been associated with a high mortality [4–7]. Planché and colleagues [4] have recently demonstrated the improved results with a single-stage repair of the aortic arch at the same time as the ASO. Despite this, the single-stage approach has not become widespread. There are very few other reports in the literature, virtually none dealing specifically with transposition of the great arteries and AAO.
We report on our experience since 1989 with a one-stage approach of complete intracardiac repair and simultaneous extensive aortic arch enlargement with pulmonary homograft patch for patients with transposition complexes and severe hypoplasia of the aortic arch.
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Patients and Methods |
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TopFootnotesAbstractIntroductionPatients and MethodsResultsCommentAddendumReferences |
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). There were 10 boys and 3 girls. All patients except 1 were less than 3 months of age, with a median age of 27 days (range, 5 days to 2 years). The median weight was 3.5 kg (range, 2.4 to 9.2 kg). All patients were taken to the operating room with the intent to do a single-stage complete repair, which was performed in 12 patients. Due to hemodynamic instability after induction of anesthesia, the second patient in the series initially underwent only extended end-to-end anastomosis and PAB through a left thoracotomy incision. Because of persistent heart failure, he successfully underwent ASO, VSD closure, and pulmonary artery debanding 12 days later at 22 days of age.
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Two patients had initially undergone PAB at another institution. One patient had TGA, VSD, and severe subaortic stenosis and received a PAB at 2 weeks of age. The baby remained in severe congestive heart failure and failure to thrive and was transferred to our institution for definitive repair. The other patient had a Taussig-Bing heart with severe subaortic stenosis, and had received a PAB in the neonatal period. At 2 years of age, with cyanosis and progressive deterioration, transfer was made to our center. Both patients were found to have AAO not previously diagnosed.
Aortic Arch Obstruction
Twelve patients had coarctation with severe aortic arch hypoplasia, fulfilling the criterion defined by the Royal Melbourne Children's Hospital group (aortic arch diameter less than the weight of the baby + 1 mm) [8].
The mean diameter of the ascending aorta for this series of patients was 7.0 ± 0.8 mm. The proximal arch (between the innominate and left carotid arteries) and distal arch (between the left carotid and left subclavian arteries) had mean diameters of 4.3 ± 0.5 mm and 3.6 ± 0.7 mm, respectively. The isthmus had a mean diameter of 2.4 ± 0.6 mm.
Great Arteries
The great artery position was anteroposterior in 3 patients, oblique in 5 patients, and side by side in 5 patients. A significant discrepancy of more than 1.5:1 was observed in most patients.
Coronary Anatomy
The coronary anatomy was usual (1LCx-2R) in 8 patients and abnormal in 5 patients [9]. Coronary distribution in 4 patients was as follows: 1RL-2Cx, 1RL-2LCx, 1R-2LCx, and 2LCxR. One patient had a bileaflet aortic valve with the right coronary from the right side of the posterior sinus and the left coronary from the left side of the posterior sinus. There were no patients with intramural coronary arteries.
Preoperative Status
Preoperative prostaglandin E1 treatment to maintain ductal patency was administered to 7 patients (54%) and a balloon atrial septostomy was performed in 5 patients (38%). Preoperative ventilatory and inotropic support was required in 4 patients (31%); 1 patient had a preoperative cardiac arrest. A fifth patient was in considerable heart failure before operation but intubation was avoided because of emergent operation.
Surgical Technique
In all patients, the repair was carried out through a sternotomy incision with the use of cardiopulmonary bypass for core cooling to deep hypothermia in preparation for circulatory arrest. Early in the series, single venous cannulation of the right atrium was used as both the aortic arch enlargement and the VSD closure were performed with two separate periods of circulatory arrest. Since 1994, double venous cannulation was used to perform the intracardiac repair on cardiopulmonary bypass, reserving circulatory arrest only for the aortic arch repair. Myocardial protection was accomplished with single dose crystalloid cardioplegia and ice-cold slush solution in the pericardial cavity was usually used. No further cardioplegia was used regardless of the length of aortic clamping. When the size discrepancy between the great vessels was marked, a U-shaped segment of anterior wall of the proximal main pulmonary artery was excised at the proposed coronary implantation sites. The coronary buttons were anastomosed with the most favorable orientation to minimize any angulation of the main coronary branches up to the neoaortic suture line. The trap-door technique was not used. VSD closure was accomplished through a ventriculotomy incision with a Gore-Tex (W. L. Gore & Assoc, Flagstaff, AZ) patch in patients with TGA, VSD. Repair of the Taussig-Bing heart required VSD closure into the pulmonary artery also with a Gore-Tex patch, followed by the ASO. The technique of aortic arch augmentation is depicted in Figure 1.
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Repair of the aortic arch was performed as follows (Fig 1B
). The undersurface of the aortic arch was opened longitudinally from the transsected distal ascending aorta, past the insertion of the ductus into the upper descending thoracic aorta for approximately 1.5 cm. The entire opened aorta was enlarged with an appropriately fashioned pulmonary homograft patch. The distal ascending aorta was thus enlarged as well as correcting the severe discrepancy often present. The neoaortic anastomosis then was constructed end-to-end without additional tailoring. All patients undergoing the ASO required the Lecompte maneuver (Fig 1C), even when the great vessels were side by side.
The mean bypass time was 224 ± 30 minutes and the mean aortic cross-clamp time was 147 ± 29 minutes. The mean circulatory arrest duration was 69 ± 26 minutes. In the last 7 patients, circulatory arrest time was reduced to 43 ± 6 minutes. Delayed sternal closure was necessary in 4 patients.
Follow-up
An annual follow-up inquiry was made to those patients who were not returning regularly to our clinic. The last inquiry was in November 1996 and all families were contacted. The mean follow-up time for all patients was 42 ± 29 months (range, 3 to 91 months).
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Results |
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TopFootnotesAbstractIntroductionPatients and MethodsResultsCommentAddendumReferences |
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Mortality
All patients survived the operation with no early mortality. One late death occurred recently at 39 months after cardiac repair from a hepatoblastoma (parents refused liver transplantation). Therefore, the total mortality for the entire series is 7.7%.
Recoarctation
The recoarctation rate is 0%. There has been no measurable gradient across the area of coarctation in any of the patients by echocardiography or by angiography in those patients who have undergone angiography at 1 to 3 years old.
Reoperations
One patient has required reoperation 21 months after repair for recurrent pulmonary stenosis and stenosis of the proximal right pulmonary artery. This patient was previously banded at another institution and had extensive scarring of the main pulmonary artery. The operation consisted of a pulmonary valvectomy and enlargement of the right ventricular outflow tract, main pulmonary artery, and proximal right pulmonary artery with a transannular pulmonary homograft patch. The postoperative course has been uncomplicated.
Morbidity
One patient suffered a cerebral infarct in the middle cerebral artery territory; he is stable neurologically at 4 years old with mild right hemiparesis and developmental delay. The patient who suffered a myocardial infarction also had a residual VSD, but now has normal ventricular function; the VSD has closed spontaneously.
Twelve patients currently have no gradient or a gradient of less than 20 mm Hg across the anastomoses of the great arteries. One patient had a mild-to-moderate obstruction at the pulmonary artery anastomosis with a gradient of 30 mm Hg by echocardiography.
Functional Status
All children are well from a cardiac point of view with a status of New York Heart Association functional class I and receiving no cardiac medications.
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Comment |
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TopFootnotesAbstractIntroductionPatients and MethodsResultsCommentAddendumReferences |
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The association of AAO with complex congenital heart disease presents a formidable surgical challenge and has been associated with a high mortality. Traditionally, these patients were repaired in two stages, with the coarctation repaired through a thoracotomy incision and the intracardiac malformation palliated with a PAB. This was followed several months or years later by debanding and intracardiac repair. For many years, this approach was not seriously questioned as some of the intracardiac repairs alone carried a high mortality. With improved results in repair of these isolated intracardiac defects, it seems that when associated with AAO, complete repair has lagged behind in success. This could be explained by several reasons. First, the association of coarctation with tubular hypoplasia of the aortic arch has only been recognized recently and complicates considerably the repair. Furthermore, this association has been reported in up to 93% of patients with complex intracardiac lesions as compared with 62% of isolated coarctation [13]. No patient in our series had isolated, discrete coarctation. Diffuse tubular hypoplasia was present in all patients. The second reason is the deleterious effects of the PAB, as have been previously described [4]. Finally, outcome assessment of the two-stage approach must take into account the mortality of the initial palliation, the interval mortality between palliation and the intracardiac repair, and the final definitive repair. Total mortality has ranged from 31% to 64% [4, 5, 7, 13].
It is on this basis that a few surgical groups, including ours, advocate single-stage repair of the complex heart defect and aortic arch through a sternotomy incision. The overwhelming majority of the reports describe the aortic arch repair with the extended end-to-end anastomosis. The overall mortality of the single-stage approach has been reported to be between 19% and 22% [4, 8, 10, 13, 14].
There are few published reports dealing specifically with AAO in patients with transposition complexes. In a recent study by the Congenital Heart Surgeons Society [5] on neonatal coarctation, overall mortality was 19% in the patients with TGA, intact ventricular septum or TGA, VSD, and 50% in those with the Taussig-Bing heart. There was no discussion, however, of the surgical approach in these patients and it must be assumed that both single-stage and two-stage approaches are included.
The single-stage repair for TGA with AAO was first reported by Pigott and associates [10] in 5 patients. There were 4 survivors. Planché and colleagues [4] demonstrated the superiority of the single-stage repair over the two-stage repair for TGA, VSD, and AAO in 1993. The two-stage group of 26 patients had an actuarial survival rate at 5 years of 57.5%. The single-stage group of 14 patients had an actuarial survival at 3 years of 78.5%. The investigators concluded that the single-stage repair of TGA, VSD, and AAO provides a better outcome than a two-stage repair. This experience was updated by Conte and associates [13] in 1995. This report includes an additional 31 patients with TGA complexes for a total experience of 71 patients. The overall mortality in the 27 patients undergoing the single-stage approach was 19%. In the 44 patients undergoing a two-stage repair, the overall mortality was 45%. Of these additional 31 patients, however, less than half received a single-stage repair. The researchers again conclude that in view of the poor results with a two-stage repair for AAO and complex cardiac anomalies, a one-stage complete repair is advocated.
Since 1989, a single-stage approach for transposition complexes with AAO has been the mainstay at our institution. Twelve of the 13 consecutive patients have undergone this complete one-stage repair. The second patient in the series, with a Taussig-Bing heart, underwent coarctation resection, extended end-to-end anastomosis, and PAB through a left thoracotomy when hemodynamic instability occurred after induction of anesthesia. Because of persistent severe congestive heart failure, the patient underwent the ASO and VSD closure 12 days after the first operation still as a neonate. The marked discrepancy between the huge proximal neoaorta and the tiny distal aorta required extensive tailoring of the anastomosis with extension into the proximal arch. This experience reinforced our conviction that the single-stage approach was superior.
Our repair of the hypoplastic aortic arch differs significantly from the resection and extended end-to-end anastomosis as performed in most centers. The transected distal ascending aorta, entire aortic arch, and the upper descending thoracic aorta is augmented with a pulmonary homograft patch as is often done during the Norwood procedure. This achieves three objectives important for adequate repair: (1) an aortic arch of adequate size, regardless of the original diameter; (2) complete correction of the often marked discrepancy between the proximal neoaorta and the distal aorta; and (3) elimination of the tension at the two aortic end-to-end anastomoses that would otherwise be necessary. This technique may potentially result in a lower incidence of recoarctation for the above mentioned reasons. The patch augmentation of the aortic arch may also play a role in the significantly improved survival in our series, by leaving no anatomic afterload that is faced by the heart after complex prolonged operation. In fact, we have not observed recoarctation in any of the 13 patients in our series. In contrast, recoarctation was observed in 4% to 20% with the resection and end-to-end anastomosis technique in the large published series including complex cardiac lesions [5, 7, 8, 13, 14].
The issue of defining aortic arch hypoplasia is by itself controversial and several definitions can be applied. We use the definition proposed by the Royal Melbourne Children's Hospital group [8], in which the aortic arch should be bigger than the baby's weight + 1 mm. If this criterion is not met, we advocate aortic arch enlargement. The importance of total relief of obstruction and thus complete afterload reduction is illustrated by our experience with a 5-week-old with TGA, VSD, and an aortic arch of 5 mm with no coarctation. The aortic arch was thought to be just adequate (weight, 3.96 kg). After ASO and VSD closure, discontinuation of cardiopulmonary bypass was achieved. But on two occasions, approximately 45 minutes later, there was rapid progressive hypotension without evidence of a coronary perfusion problem. Cardiac arrest ensued requiring reinstitution of cardiopulmonary bypass. External examination revealed full coronary arteries and no region of myocardial ischemia. The cause was believed to be a borderline hypoplastic aortic arch. Both pulmonary and aortic anastomoses were taken down, the aortic arch was augmented with a pulmonary homograft patch, and the aortic and pulmonary anastomoses were reconstructed. The patient recovered successfully.
Subaortic obstruction is present in a significant number of these patients. Although this becomes the subpulmonary area after the ASO, it must be adequately relieved to achieve a good outcome. The nature of subaortic obstruction was muscular obstruction in the subaortic infundibulum; it was corrected by resection and pericardial patch augmentation of the ventriculotomy incision. In 1 patient with the Taussig-Bing heart, the subaortic area was only 2 mm and a transannular patch was not possible due to a right coronary artery arising from the left anterior descending artery and crossing the subaortic area. In this patient, biventricular repair was achieved with an operation borrowing features of the Rastelli, Damus-Kaye-Stansel, and Norwood operations. The left ventricular blood was tunnelled through the VSD into the proximal main pulmonary artery, which was then transected to be incorporated into the systemic circulation. The aortic arch was then augmented with a pulmonary homograft patch as previously described and the systemic circulation is brought over the proximal main pulmonary artery. Finally, right ventricle to distal pulmonary artery continuity was completed with a pulmonary homograft. The narrow subaortic area was oversewn.
Our results have been very encouraging. One patient, asymptomatic from a cardiac point of view, developed a hepatoblastoma and died 39 months after the cardiac repair as the parents turned down a liver transplantation. The other 12 patients are in good cardiac condition in New York Heart Association functional class I. There has been one reoperation for right ventricular outflow tract obstruction and right pulmonary artery stenosis with excellent recovery. This patient had received a previous PAB.
In conclusion, single-stage repair for TGA complexes with AAO achieves excellent survival, significantly higher than the reported survival with a two-stage approach. It should be considered the surgical approach of choice for virtually all patients, with the possible exception of multiple surgically inaccessible VSDs or those associated with significant ventricular hypoplasia. We believe that pulmonary homograft patch augmentation of the hypoplastic aortic arch achieves superior relief of anatomic afterload, correction of the frequently observed great vessel mismatch with tension-free anastomosis and a lower recoarctation rate.
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Addendum |
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TopFootnotesAbstractIntroductionPatients and MethodsResultsCommentAddendumReferences |
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Footnotes |
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TopFootnotesAbstractIntroductionPatients and MethodsResultsCommentAddendumReferences |
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Address reprint requests to Dr Tchervenkov, Cardiovascular Surgery, The Montreal Children's Hospital, 2300 Tupper, C-827, Montreal, PQ, Canada, H3H 1P3.
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References |
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TopFootnotesAbstractIntroductionPatients and MethodsResultsCommentAddendumReferences |
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