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J Thorac Cardiovasc Surg 2001;122:524-528
© 2001 The American Association for Thoracic Surgery
Surgery for Congenital Heart Disease (CHD) |
From the Department of Pediatric Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical University, Tokyo, Japan.
Received for publication Oct 10, 2000. Revisions requested Jan 16, 2001; revisions received Feb 16, 2001. Accepted for publication Feb 26, 2001. Address for reprints: Yukihisa Isomatsu, MD, Department of Pediatric Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical University, 8-1 Kawadacho, Shinjuku-ku, Tokyo 162-8666, Japan.
Abstract
Background: Optimal management for coarctation of the aorta and ventricular septal defect remains controversial. The current study was undertaken to determine outcome, including recoarctation after 2-stage repair, at our institution.
Methods: Between 1984 and 1998, 79 patients younger than 3 months with coarctation and ventricular septal defect underwent 2-stage repair at our institution. The first-stage operation consisted of subclavian flap angioplasty and pulmonary banding. The median age at the time of first operation was 28 days (range, 4-90 days), and median weight was 3.2 kg (range, 1.2-5.1 kg). Hypoplastic aortic arch was present in 27 patients, and coexisting anomalies were present in 13 patients. After a mean interval of 10.4 ± 9.6 months, they underwent a second-stage repair, with closure of the ventricular septal defect and pulmonary debanding.
Results: There were 2 hospital deaths and 4 late deaths. Mean follow-up was 9.2 ± 4.9 years (range, 2.0-18.3 years), and actuarial survival was 92.3% at 10 years (95% confidence interval, 86.6%-98.3%). Age at first operation, body weight, hypoplastic arch, and coexisting anomalies had no significant influence on overall mortality. Freedom from recoarctation rate was 90.4% at 10 years (95% confidence interval, 83.7%-97.2%).
Conclusion: To diminish mortality and the recoarctation rate and also to decrease the possibility of complications related to circulatory arrest and allogeneic blood transfusion, 2-stage repair is still an effective technique for coarctation of the aorta associated with ventricular septal defect.
See related editorial on page 424.
The optimal management for patients with coarctation of the aorta and ventricular septal defect (VSD) remains controversial. The traditional 2-stage operation consists of the repair of coarctation with or without pulmonary banding through a left thoracotomy and the closure of VSD as a secondary procedure. On the other hand, both coarctation and VSD are repaired at the same time in a single-stage repair. According to a multi-institutional study in 1994,
1 single-stage repair had the lowest survival, whereas 2-stage repair concomitant with pulmonary artery banding (PAB) had the highest survival. However, recent studies 2,3 have reported good results for a primary total correction procedure through a midline sternotomy. With 2 surgical options now available, a major concern is which procedure has the relative benefit, the single-stage versus the 2-stage repair for coarctation and VSD. This study was undertaken to determine the outcome, including recoarctation, after so-called traditional 2-stage repair at our institution.
Methods
Between 1984 and 1998, 79 patients younger than 3 months who had coarctation of the aorta and VSD were operated on at the Heart Institute of Japan (Tokyo Women's Medical University, Japan). Patients with transposition of the great arteries, double-outlet right ventricle, univentricular heart, atrioventricular septal defect, tricuspid atresia, and Ebstein anomaly were excluded from the study. Patients with interrupted aortic arch were also excluded. The medical records of all these infants were retrospectively reviewed. The diagnosis of coarctation and VSD, the location of VSD, and the presence of any coexisting anomalies were determined by echocardiography, cardiac catheterization, and operative records. In the latter part of this period, cardiac catheterization was performed only when the echocardiographic diagnosis was not conclusive.
Patients
There were 52 (66%) boys and 27 (44%) girls among 79 patients with coarctation and VSD under 3 months of age. The median age at the time of operation was 28 days (range, 4-90 days; mean, 37 days), and median weight at the time of operation was 3.2 kg (range, 1.2-5.1 kg; mean, 3.3 kg). Six patients had ductal shock and the other 73 patients had congestive heart failure as the primary indication for operation. Alprostadil (prostaglandin) infusion before the operation was used in 58 (73%) patients. Hypoplastic arches, defined as narrowest arch diameter of 4 mm or less, 4 were observed in 27 (34%) patients. The location of the VSD was perimembranous in 47 (59%) patients, subpulmonary in 28 (35%) patients, and muscular in 4 (5.1%) patients. All 4 patients with a muscular VSD had other perimembranous VSDs (multiple VSDs). Coexisting anomalies were subaortic stenosis in 5 (6.3%) patients, aortic valve stenosis in 4 (5.1%) patients, bicuspid aortic valve in 3 (3.8%) patients, and aortic valve regurgitation in 1 (1.3%) patient.
Surgical procedures
Our consistent surgical policy for coarctation and VSD has been a 2-stage repair: coarctation repair by means of subclavian flap angioplasty (SFA) 5 concomitant with PAB 6 as a first-stage procedure and closure of VSD associated with debanding as a second-stage procedure.
All patients underwent SFA and PAB through a left lateral thoracotomy. After heparinization, the left subclavian artery was well mobilized by dividing all branches, and the ductus arteriosus was ligated. The subclavian artery was opened downward into the descending aorta well beyond the coarctation segment, and the aorta was enlarged with the subclavian flap with 7-0 Prolene sutures before or 7-0 PDS running sutures (Ethicon, Inc, Somerville, NJ) after July 1987. The pericardium was then incised, and the pulmonary artery was isolated with a 3-mm wide Teflon tape at the level of bifurcation. The pulmonary artery was usually banded down to a circumference of 19 mm plus the patient's body weight in kilograms. The band was secured to the adventitia of the pulmonary artery at several portions to prevent migration and distortion of the pulmonary artery. Hypoplastic aortic arches were left undisturbed on the basis of our belief that adequate aortic forward flow is a major factor in the arch development in this disease. One patient required a second PAB because he could not be weaned from the ventilator.
After a mean interval of 10.4 ± 9.6 months (range, 9 days-29 months), a second-stage operation was performed through a median sternotomy, and bicaval cannulation with hypothermic cardiopulmonary bypass was performed. After total bypass was established, the main pulmonary artery was transected at the band site, and pulmonary debanding was performed by excising the banded segment of the main pulmonary artery. The VSD was closed with a Dacron patch under cardiac arrest induced with crystalloid cardioplegia. In one patient plication of the right coronary cusp for aortic insufficiency was performed during the same operation. Pulmonary artery continuity was restored by means of an end-to-end direct anastomosis. The mean duration of cardiopulmonary bypass was 99 ± 30 minutes, and the mean duration of aortic crossclamping was 39 ± 28 minutes.
All patients were followed up at our outpatient unit, and follow-up was complete and up to date in all patients.
Statistical analysis
Data computations were performed with the StatView 5.0 (SAS Institute Inc, Cary, NC) statistical program. Proportion values are expressed as mean values with 95% confidence intervals (CI). Continuous data are presented as means ± standard deviation (SD). Time-related events were examined by Kaplan-Meier actuarial methods. Preoperative factors associated with the overall mortality rate or the recoarctation rate were assessed by multivariate analysis with a Cox proportional hazards regression model. For analyses of both survival and freedom from recoarctation, the time of the initial operation was taken as time zero.
Results
Survival
There were 2 hospital deaths (mean, 2.5%; 95% CI, 0.01%-6.0%). All hospital mortalities were related to the initial operation performed to repair the coarctation. One patient (15 days of age), who preoperatively had necrotizing enterocolitis caused by ductal shock, died of sepsis 5 days after the operation. The second patient (18 days of age) died 26 days after the operation because of heart failure caused by hypertrophic cardiomyopathy, which was diagnosed by means of echocardiography that revealed concentric hypertrophy of the left ventricle. There were 4 late deaths (mean, 5.2%; 95% CI, 0.2%-10.1%). Cause of death was low output syndrome 7 hours after the second operation in one patient who had a concomitant infundibular resection. The second patient had SFA and PAB at 12 days of age and peritoneal dialysis at 23 days of age caused by idiopathic hyperbilirubinemia and underwent a second-stage operation 26 days after the first operation. He was unable to be weaned from the ventilator, showed pulmonary infection and sepsis, and died 34 days after the second operation. The third patient died of left ventricular failure that was unresponsive to pharmacologic treatment 9 months after the second-stage operation. The final patient died of rapidly progressive congestive heart failure at 32 months after the second-stage operation. Mean follow-up in all 73 late survivors was 9.2 ± 4.9 years (range, 2.0-18.3 years), and the actuarial survival was 92.3% at 10 years (95% CI, 86.6%-98.3%; Figure 1). In multivariate analysis, age at the first operation, body weight at the first operation, the presence of hypoplastic arch, and coexisting anomalies had no significant influence on overall mortality (P = .707, P = .137, P = .466, and P = .510, respectively;Table 1).
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Single-stage repair has 3 potential disadvantages when compared with a 2-stage repair: (1) There is a high mortality rate, recoarctation rate, or both; (2) deep hypothermic circulatory arrest is necessary; and (3) allogeneic blood transfusion is required(Table 2).
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7 reported that hospital mortality was 4.7% in the multistage group and 18.8% for the single-stage group, although this difference did not reach statistical significance. The actuarial survival at 10 years after the 2-stage operation was reported to be 96%, 94%, 92.6%, and 91%(Table 3). 1-3,7-12 These values are similar to our results. On the other hand, the survival after single-stage repair was reported to be 80.0% at 2 years, 81.2% at 5 years, 91.0% at 1 year, and 96.0% at 3 years(Table 3).
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7 Our results, with a 90.4% freedom from recoarctation rate at 10 years after 2-stage repair, compare favorably with this figure. Freedom from recoarctation was 75% in another study, even at 2 years after single-stage repair. 3 These lower rates of freedom from recoarctation compared with 2-stage repair suggest that, through a sternotomy, ductal tissue was inadequately removed to avoid the left bronchial compression 13 by the reconstructed aortic arch. This might result in frequent stenosis in the repair area.
Regarding which procedure has better results, SFA or end-to-end anastomosis, many surgeons may prefer resection and end-to-end, although there is little convincing data from comparisons of one type of repair with another. This decision is based primarily on the belief that the coarctation segment containing abnormal ductal tissue should be resected to prevent recurrence. 14 It is true that ductal tissue is partially excised, and residual ductal tissue is left behind in the SFA technique. However, Merril and colleagues 10 demonstrated that the recoarctation rate was better for SFA than that for end-to-end in patients who were followed up for at least 5 years. It was also pointed out by Gaynor and associates 3 that increased tension at the anastomosis caused by end-to-end might result in a high possibility of anastomotic stenosis. They used a homograft patch augmentation technique for arch reconstruction in 21 of 25 patients who underwent single-stage repair.
Deep hypothermic circulatory arrest is commonly used during not only aortic arch repair through a median sternotomy but also during arterial switch operations in neonates in many centers. It is a widespread concept that brain damage caused by systemic hypoxia-hypotension is at least reduced by induction of hypothermia. However, there is evidence that neurologic injury could occur, which might lead to transient cerebral dysfunction 15,16 and delayed psychomotor development. 17 Only exceptional surgeons 2,11,18 advocated the use of an isolated cerebral perfusion from the right brachiocephalic artery during arch repair. However, there is not definite proof that brain circulation is normally maintained because the circle of Willis is hypoplastic or incomplete in more than 70% of human subjects. 19,20
Allogeneic blood transfusion is indispensable for single-stage repair under cardiopulmonary bypass, especially in neonates, although the possible risk of transfusion-transmitted diseases have been of particular concern in cardiac surgery. When choosing a 2-stage repair, it is possible to complete procedures without blood transfusion through stages. In fact, 8 patients underwent 2-stage repair without allogeneic blood transfusion since 1995 of a total of 13 patients who reached the second stage.
Disadvantages in our 2-stage repair are as follows: (1) such a strategy requires that all infants undergo a second operation for VSD closure and pulmonary artery debanding and (2) the left subclavian artery must be sacrificed. We believe that the staged strategy is justified by a good survival rate and higher freedom from low incidence of recoarctation after 2-stage repair compared with that after single-stage repair. We also believe that vascular insufficiency of the left arm is quite unusual because we have no experience of a need for amputation or a need for medical-surgical treatment after SFA. We think the relative benefits for patients with coarctation and VSD, at present, is clear when weighing disadvantages in both 2-stage repair and single-stage repair.
In conclusion, to minimize mortality and recoarctation rate and also to decrease the possibility of complications related to circulatory arrest and allogeneic blood transfusion, so-called traditional 2-stage repair, to date, is still an effective technique for coarctation associated with VSD. It will not be until there are better results regarding, at a minimum, survival rate and freedom from recoarctation that single-stage repair will be conceived to be the choice of treatment. We believe that SFA and concomitant PAB performed properly as a first-stage repair are important for better long-term results in patients with coarctation with VSD.
Acknowledgments
We thank Dr Nakamura Tsuyoshi for his contribution to the statistical analysis.
References
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