|
|
||||||||
J Thorac Cardiovasc Surg 2002;124:790-797
© 2002 The American Association for Thoracic Surgery
Surgery for Congenital Heart Disease (CHD) |
From the Children's Heart Center of the Wilhelmina Children's Hospital, University Medical Center, Utrecht, The Netherlands.
Received for publication July 2, 2001. Revisions requested Aug 30, 2001; revisions received Sept 20, 2001. Accepted for publication Sept 9, 2001. Address for reprints: P. A. Hutter, Wilhelmina Children's Hospital, Room No. KG.01.319.0, PO Box 85090, 3508 AB, Utrecht, The Netherlands (E-mail: p.hutter{at}wkz.azu.nl).
| Abstract |
|---|
|
|
|---|
| Introduction |
|---|
|
|
|---|
Twenty-five years ago Jatene and colleagues
1 first described the arterial switch operation (ASO) for transposition of the great arteries (TGA). The operation seemed a simple solution for a significant problem. The ASO technique was more complex than its predecessors, the Mustard and Senning procedures,
2,3 and it required some time before the technique became widely accepted. All the prerequisites for a successful (neonatal) ASO were recognized in time and dealt with, which allowed general acceptation of the technique in the 1980s.
4 Mortality has decreased as expertise has been gained and modifications have been introduced to tackle the problem of supravalvular pulmonary stenosis.
5 Twenty-five years have passed since the first ASO was performed in Brazil, and 24 years have passed since the first operation was done in our institution. This study reports on the results and the follow-up to date.
| Patients and methods |
|---|
|
|
|---|
|
Pulmonary stenosis suspected on the basis of Doppler echocardiography was always verified by cardiac catheterization (gradient >50 mm Hg or right ventricular pressure >75% of systemic pressure). The aortic root was evaluated by Doppler echocardiography.
8
Left ventricular dysfunction was defined as a shortening fraction less than 27% on the parasternal long axis. Invasive confirmation was always obtained.
Development of the switch technique
Initially, 30 patients underwent a two-stage procedure after pulmonary artery banding. When cyanosis was severe, a 5-mm aortopulmonary shunt was added (n = 11 patients). One hundred sixty-four patients underwent primary ASOs. Neonatal switch has been preferred for simple TGA since 1984 and for TGA with ventricular septal defect (VSD) since 1986.
The ASO was performed according to Jatene and colleagues' original technique, with a conduit when necessary to establish continuity of the pulmonary artery in 9 patients before 1982.
9 With sufficient length of the pulmonary artery, the gaps left by the coronary arteries were filled with glutaraldehyde-treated pericardium.
10 From 1980 onward, the maneuver described by Lecompte and associates
5 was performed.
From 1983 to 1991, a total of 9 patients had a button patch in one ostium and a "hemipantaloon" of treated pericardium in the other ostium to overcome traction on the anastomosis. A pantaloon-shaped patch of fresh autologous pericardium was used starting in 1991.
11
Statistical analysis
To investigate survival and time to reintervention or arrhythmia, the Kaplan-Meier method was used with log-rank tests and Cox regression models to test for independence of covariates. Logistic regression was used in the analysis of early mortality. Univariate analyses were performed with the Student t test and
2 test.
| Results |
|---|
|
|
|---|
|
Most patients (n = 145) are in New York Heart Association (NYHA) functional class I. Four patients are in NYHA functional class II. Among these 4, the first patient had left ventricular dysfunction after an ASO at the age of 1 day. Intensive care course was complicated by junctional ectopic tachycardia with repeated episodes of ventricular fibrillation. The coronary arteries seemed normal, as confirmed by later coronary angiography. This patient had left ventricular dilatation and severe aortic insufficiency. The aortic valve was replaced. The patient still has severe left ventricular dysfunction, has limited exercise capacity, and continues to receive medication. A second patient had an occluded left coronary artery and a myocardial infarct after the operation. Ventricular fibrillation developed at the age of 15 years. An automatic defibrillator was implanted, and he is being treated with ß-blockers. The third patient was banded as a neonate but did not undergo the switch before the age of 5 years for reasons related to his status as a child of political refugees. His left ventricle never recovered from loss of muscle mass, and he is dependent on medication. Finally, the fourth patient acquired atrial flutter, for which he receives medication. He is now symptom free with good exercise tolerance.
Reinterventions
Sixty reinterventions were necessary in 35 patients (Table 3). Figure 1 demonstrates freedom from reintervention.
|
|
Among the 125 patients who did not have a reintervention to relieve pulmonary stenosis, the highest flow velocity on Doppler echocardiography in the main pulmonary artery or the branches was 2.3 ± 0.6 m/s (calculated gradient 23 ± 12 mm Hg) at the latest follow-up.
The pulmonary artery was reconstructed with a conduit in 5 patients, with two separate button patches in 62, and with a pantaloon-shaped patch in 85. The Lecompte maneuver was used in 131 patients.
In the Cox regression model, the method of pulmonary reconstruction was the only independent predictor of pulmonary stenosis necessitating reintervention (Figure 2, P = .011). On further analysis, use of a conduit was the determining factor. In a model without the patients who had conduits, it was the material of the patch (glutaraldehyde-treated pericardium or fresh pericardium, P = .02), rather than the shape (buttons or pantaloon), that was the determining factor (P = .84).
|
Sixteen patients needed a single reintervention for pulmonary stenosis; this intervention was surgical in 9 cases and by balloon angioplasty in 7. Six patients required two procedures (catheterization followed by an operation in 3 cases, two catheterizations in 2 cases, and two operations in 1 case). In 4 patients three or more procedures were necessary, starting with surgical intervention in all cases. In cases of valvular of subvalvular stenosis, catheter interventions were unsuccessful. Stenosis in the distal main pulmonary artery and in the branches is more amenable to balloon angioplasty, although some of our patients will need stent implantation in the future. Surgery consisted of patch angioplasty of the main pulmonary artery or the branches in 13 procedures. A transannular patch was necessary in 8 patients, and 2 patients underwent homograft replacement of the pulmonary valve.
Arrhythmias
Five patients had arrhythmias develop during follow-up (Figure 3). Two had ventricular fibrillation, and 1 of these died. In the other patient a defibrillator was implanted at the age of 15 years. Both patients had perioperative myocardial infarctions and left coronary obstruction. A third patient had sick sinus syndrome without adequate ventricular escape. He had TGA with VSD and underwent the switch at the age of 57 days (after a Rashkind procedure) and received a pacemaker at the age of 12 years. The fourth patient had a single attack of supraventricular tachycardia at the age of 11 years. No medication was prescribed. The fifth patient had severe pulmonary stenosis with tricuspid insufficiency. He withdrew from follow-up for 6 years and subsequently underwent reoperation. Three years later he had atrial flutter. He underwent cardioversion and is maintained with medication pending ablation of the flutter circuit.
|
The flow velocity measured by Doppler echocardiography in the ascending aorta was a mean of 1.32 m/s, without a significant increase with time (P = .14). At the latest follow-up, aortic flow velocity was less than 2 m/s (<16 mm Hg) in 137 patients, 2 to 3 m/s (16-36 mm Hg) in 13 patients, and 3.2 m/s (41 mm Hg) in 1 patient. Stenosis of the aortic valve or anastomosis leading to left ventricular hypertrophy on ECG or echocardiography did not occur. One patient had mild stenosis develop at the aortic anastomosis, without left ventricular hypertrophy. He required reintervention for pulmonary stenosis, and patch angioplasty of the aorta was performed during this procedure. One patient had TGA with subpulmonary VSD and left ventricular outflow tract stenosis, thought to be caused by bulging of the septum because of the higher right ventricular pressure. After ASO, however, the stenosis increased, necessitation reoperation half a year later. The patient with interrupted aortic arch had severe stenosis develop at the site of aortic cannulation; this necessitated reoperation within the first postoperative week.
Coronary arteries
To date, cardiac catheterization has been performed in 61 patients who were not selected for symptoms or abnormal findings on ECG or echocardiography. Coronary sequelae were found in 5 patients. There was no relationship to the initial coronary anatomy.
12 Two patients had an occluded left coronary artery (Figure 4); both had ventricular fibrillation develop as described previously. Whether an attempt should be made to reperfuse the occluded coronary artery in the surviving patient is still under debate.
|
Asymptomatic stenosis of the right coronary ostium was documented in a fourth patient, and a fifth patient has multiple tortuous collaterals without evidence of obstruction of a major coronary branch. These last 2 patients have normal 12-lead and 24-hour ECGs, normal left ventricular function, and normal scintiscans.
Left ventricular dysfunction
Left ventricular dysfunction was encountered in 5 patients. Two patients had occluded left coronaries as described previously. In another patient the left ventricular dysfunction was attributed to failure of myocardial protection during the switch. The fourth patient had loss of left ventricular mass related to a very late ASO, despite pulmonary artery banding as a neonate. This patient had a protracted postoperative course and still requires medication. Finally, 1 patient had enlargement of the left ventricle with suboptimal contractility, without coronary artery abnormalities. This patient had Taussig-Bing anomaly and had undergone coarctectomy and banding of the pulmonary artery as a neonate. An ASO was performed at the age of 1.4 years. Loss of muscle mass before the switch is a possible factor, although his postoperative course was uneventful.
Two-stage switch
A two-stage ASO was performed in 31 cases. Thirty patients underwent banding, combined with a shunt in 11 cases. One patient underwent a shunt without banding because of hypoplastic pulmonary branches.
Early mortality in this group was 9 patients (29%). In univariate analysis, banding was a risk factor for early death (P = .025), but in the multivariate analysis surgical experience rather than banding was the significant factor.
In the group of patients with late complications, many had previous banding; only 8 of 22 patients surviving this two-stage procedure did not have any complications (Table 4). In Cox regression analysis, banding (P = .05) and shunt (P = .02) were independent predictors for one or more postoperative complications.
|
| Discussion |
|---|
|
|
|---|
In our study population, the initial perioperative mortality was as high as 60%, leading to discussions about whether to continue implementing this technique.
16 Gradually, improving results of the neonatal ASO provided the support necessary for us to persevere. Like other groups, we are now able to report acceptable mortalities.
17-19
Late mortality has been limited to 2 patients. One patient with simple TGA died of irreversible pulmonary hypertension, even though he had never been exposed to elevated pulmonary arterial pressures. The development of pulmonary vascular disease is well documented in patients with TGA and VSD but less well known in patients with simple TGA. In 1998, Rivenes and Grifka
20 reported on a small group of patients who, despite adequate and timely intervention, had pulmonary vascular occlusive disease develop. This spontaneous development of pulmonary vascular disease in TGA, although rare, remains a significant unsolved problem.
The other late death was that of a patient with postoperative coronary pathologic changes. Perioperative left ventricular ischemia and dysfunction was the main cause of early mortality in our series. Postoperative coronary pathologic changes may be seen late, without perioperative complications, as reported by Bonhoeffer and coworkers,
6 by Tanel and associates,
7 and by our group.
12 The percentage of patients with such changes is small, but the implications are serious. Pathologic changes can be seen late, with ventricular arrhythmia or sudden death after a long silent interval of many years. As Bonhoeffer and coworkers
6 advocated, we have adopted a policy to perform coronary angiography in the follow-up of all ASOs, currently at the age of 10 years. Whether patients are at increased risk for ischemic heart disease later in life remains to be determined.
Arrhythmias are related in part to imperfections in the ASO: coronary occlusion with infarction or long-standing right ventricular pressure overload from pulmonary stenosis. The precise causes of the binodal disease (sick sinus syndrome without ventricular escape) and of the single episode of supraventricular tachycardia are unclear, and these conditions are not necessarily attributable to the surgery. In a comparison of the incidence of supraventricular arrhythmias after switch operations with the incidence after atrial baffle procedures, the advantage of the ASO is overwhelmingly clear.
21
A two-stage ASO was the norm during the early experience. Some lessons can be learned concerning retraining the left ventricle for a late ASO in the current era. Loss of left ventricular muscle mass may lead to higher perioperative mortality risk, even if the pulmonary band seems adequate. Persistent left ventricular dysfunction and late arrhythmias should be anticipated.
Significant aortic insufficiency is rare and related to the original anatomy, especially subpulmonary VSDs. Lack of support of the overriding pulmonary (neoaortic) valve may be an important factor. Size difference between the great arteries, often more pronounced in this type of VSD, is another possible cause. As we have previously reported elsewhere, progressive dilatation of the neoaortic root did not seem to be a factor.
8 A significant relationship with pulmonary artery banding, as was previously found by others,
22 was not encountered.
Stenosis of the aortic anastomosis is seldom seen after the arterial switch because, unlike with the pulmonary anastomosis, there is no traction. Also, a pulmonary (neoaortic) valve of insufficient size will preclude an ASO, whereas stenosis of the right ventricular outflow tract is no contraindication.
Most surviving patients did not have problems and are in NYHA class I, not unlike the experience that Kirklin and colleagues
23 reported in 1992. Four of our patients are in NYHA class II, with left ventricular dysfunction, arrhythmias, or both.
Pulmonary stenosis remains the most frequent complication and the most frequent cause of reintervention. The continuous modifications of ASO have decreased the incidence of pulmonary stenosis. However, insufficient follow-up duration does preclude interpretation of improvement through use of the pantaloon patch. Currently, the pulmonary branches are dissected down to the hilum, and an ample pantaloon patch is used to elongate the main pulmonary artery. The incidence of pulmonary stenosis is with this technique expected to drop well below 10%. Some pulmonary stenosis will remain in patients with unfavorable anatomy, such as our patient with hypoplastic pulmonary branches.
The overall outcome of this group of patients is promising and supports the decision to abandon the atrial switch procedure. However, an in-depth study of quality of life and school and professional careers, like those that have been performed for patients after Mustard and Senning operations,
24 has not yet been performed for patients after the ASO. To complete the long-term evaluation of the ASO, such a study seems imperative.
| Conclusion |
|---|
|
|
|---|
| References |
|---|
|
|
|---|
Related Article
This article has been cited by other articles:
![]() |
C. A. Warnes, R. G. Williams, T. M. Bashore, J. S. Child, H. M. Connolly, J. A. Dearani, P. del Nido, J. W. Fasules, T. P. Graham Jr, Z. M. Hijazi, et al. ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease) Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons J. Am. Coll. Cardiol., December 2, 2008; 52(23): e1 - e121. [Full Text] [PDF] |
||||
![]() |
C. A. Warnes, R. G. Williams, T. M. Bashore, J. S. Child, H. M. Connolly, J. A. Dearani, P. del Nido, J. W. Fasules, T. P. Graham Jr, Z. M. Hijazi, et al. ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease): Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons Circulation, December 2, 2008; 118(23): e714 - e833. [Full Text] [PDF] |
||||
![]() |
J. Skinner, T. Hornung, and E. Rumball Transposition of the great arteries: from fetus to adult Heart, September 1, 2008; 94(9): 1227 - 1235. [Full Text] [PDF] |
||||
![]() |
A. Yamazaki, N. Yamamoto, T. Sakamoto, K. Ishihara, Y. Iwata, G. Matsumura, and H. Kurosawa Long-term outcomes and social independence level after arterial switch operation Eur. J. Cardiothorac. Surg., February 1, 2008; 33(2): 239 - 243. [Abstract] [Full Text] [PDF] |
||||
![]() |
J. Horer, C. Schreiber, E. Dworak, J. Cleuziou, Z. Prodan, M. Vogt, K. Holper, and R. Lange Long-Term Results After the Rastelli Repair for Transposition of the Great Arteries Ann. Thorac. Surg., June 1, 2007; 83(6): 2169 - 2175. [Abstract] [Full Text] [PDF] |
||||
![]() |
D. H. Freed, C. M.T. Robertson, R. S. Sauve, A. R. Joffe, I. M. Rebeyka, D. B. Ross, J. D. Dyck, and the Western Canadian Complex Pediatric Therapies P Intermediate-term outcomes of the arterial switch operation for transposition of great arteries in neonates: Alive but well? J. Thorac. Cardiovasc. Surg., October 1, 2006; 132(4): 845 - 852. [Abstract] [Full Text] [PDF] |
||||
![]() |
G. E. Sarris, A. C. Chatzis, N. M. Giannopoulos, G. Kirvassilis, H. Berggren, M. Hazekamp, T. Carrel, J. V. Comas, D. Di Carlo, W. Daenen, et al. The arterial switch operation in Europe for transposition of the great arteries: A multi-institutional study from the European Congenital Heart Surgeons Association. J. Thorac. Cardiovasc. Surg., September 1, 2006; 132(3): 633 - 639. [Abstract] [Full Text] [PDF] |
||||
![]() |
M. V. Ullmann, M. Gorenflo, C. Bolenz, C. Sebening, M. Goetze, R. Arnold, H. E. Ulmer, and S. Hagl Late Results After Extended Pulmonary Artery Reconstruction in the Arterial Switch Operation Ann. Thorac. Surg., June 1, 2006; 81(6): 2259 - 2266. [Abstract] [Full Text] [PDF] |
||||
![]() |
I. A. Russell, K. Rouine-Rapp, G. Stratmann, and W. C. Miller-Hance Congenital heart disease in the adult: a review with internet-accessible transesophageal echocardiographic images. Anesth. Analg., March 1, 2006; 102(3): 694 - 723. [Full Text] [PDF] |
||||
![]() |
Y. H. Chang, S. C. Sung, H. D. Lee, S. Kim, J. S. Woo, and Y. S. Lee Coronary Reimplantation After Neoaortic Reconstruction Can Yield Better Result in Arterial Switch Operation: Comparison With Open Trap Door Technique Ann. Thorac. Surg., November 1, 2005; 80(5): 1634 - 1640. [Abstract] [Full Text] [PDF] |
||||
![]() |
S. G Raja, A. Shauq, and M. Kaarne Outcomes after Arterial Switch Operation for Simple Transposition Asian Cardiovasc Thorac Ann, June 1, 2005; 13(2): 190 - 198. [Abstract] [Full Text] [PDF] |
||||
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
| ANN THORAC SURG | ASIAN CARDIOVASC THORAC ANN | EUR J CARDIOTHORAC SURG |
| J THORAC CARDIOVASC SURG | ICVTS | ALL CTSNet JOURNALS |