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J Am Coll Cardiol, 1999; 33:1702-1709 © 1999 by the American College of Cardiology Foundation |






* Department of Cardiology, Childrens Hospital, Boston, Massachusetts, USA
Department of Cardiac Surgery, Childrens Hospital, Boston, Massachusetts, USA
Department of Pediatrics and Department of Surgery, Harvard Medical School, Boston, Massachusetts, USA
Current affiliation: Columbia Presbyterian Medical Center, New York, New York, USA
Reprint requests and correspondence: Dr. Tal Geva, Department of Cardiology, Childrens Hospital, 300 Longwood Avenue, Boston, Massachusetts 02115
geva_t{at}a1.tch.harvard.edu
| Abstract |
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The present study was undertaken to determine the independent risk factors for early mortality in the current era after arterial switch operation (ASO).
BACKGROUND
Prior reports on factors affecting outcome of the ASO demonstrated that abnormal coronary arterial patterns were associated with increased risk of early mortality. As diagnostic, surgical and perioperative management techniques continue to evolve, the risk factors for the ASO may have changed.
METHODS
All patients who underwent the ASO at Childrens Hospital, Boston between January 1, 1992 and December 31, 1996 were included. Hospital charts, echocardiographic and cardiac catheterization data and operative reports of all patients were reviewed. Demographics and preoperative, intraoperative and postoperative variables were recorded.
RESULTS
Of the 223 patients included in the study (median age at ASO = 6 days and median weight = 3.5 kg), 26 patients had aortic arch obstruction or interruption, 12 had Taussig-Bing anomaly, 12 had multiple ventricular septal defects, 8 had right ventricular hypoplasia and 6 were premature. There were 16 early deaths (7%), with 3 deaths in the 109 patients considered "low risk" (2.7%). Coronary artery pattern was not associated with an increased risk of death. Compared with usual coronary anatomy pattern, however, inverted coronary patterns and single right coronary patterns were associated with increased incidence of delayed sternal closure (p = 0.003) and longer duration of mechanical ventilation (p = 0.008). In a multivariate logistic regression model using only preoperative variables, aortic arch repair at a separate procedure before ASO and smaller birth weight were independent predictors of early mortality. In a second model that included both pre- and intraoperative variables, circulatory arrest time and right ventricular hypoplasia were independent predictors of early death.
CONCLUSIONS
The ASO can be performed in the current era without excess early mortality related to uncommon coronary artery patterns. Aortic arch repair before ASO, right ventricular hypoplasia, lower birth weight and longer intraoperative support continue to be independent risk factors for early mortality after the ASO.
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With increasing experience, improving perioperative outcomes and reassuring results of medium-term follow-up (1,2,1115), patients with increasingly complex cardiovascular anatomy are undergoing ASO (1620). Included are patients with DORV, left and right ventricular outflow obstruction, multiple ventricular septal defects (VSD), anomalies of the atrioventricular canal and valves and aortic arch anomalies. As the application of the ASO to patients with increasingly complex anatomy and hemodynamics broadens and as methods for diagnosis and management of these infants evolve, it is likely that the risk factors for this operation also change. Specifically, our clinical observations suggest that in recent years, coronary artery pattern may not be associated with increased risk of early mortality after the ASO. Mayer and colleagues (21) speculated that although coronary artery pattern was an overall risk factor for early mortality, its impact over time would diminish. The present study, therefore, was designed to examine the hypothesis that coronary artery pattern is no longer an independent risk factor of early mortality, and to determine the independent risk factors in the current era for early mortality after ASO.
| Methods |
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Data collection. Patient variables included demographic information, prematurity (gestational age <37 weeks), birth weight, age, weight and body surface area at surgery and associated noncardiac malformations. The following anatomic variables were recorded: presence or absence of VSDpatients were categorized as having an intact ventricular septum if no VSD or a tiny defect was documented by a preoperative echocardiogram and/or catheterization, and VSD was defined as a defect judged to be sufficiently large to warrant surgical closure at the time of ASO; the presence of multiple VSDs; qualitative assessment of the position of the great arteries relative to each other; right ventricular hypoplasia (defined as tricuspid valve annulus z-score <2 or a nonapex-forming right ventricle), and aortic arch anomalies including interruption, coarctation or arch hypoplasia. Coronary anatomy was determined by direct visualization at the time of surgery. Coronary artery patterns were classified as previously published (2123) and are outlined in Figure 1. Both the individual and grouped patterns were analyzed. Preoperative data included balloon atrial septostomy, prior cardiac surgical procedures and the use of extracorporeal support. Intraoperative procedural data recorded included surgical techniques such as Lecompte maneuver, VSD closure, aortic arch repair and right ventriculotomy, as well as takedown of a prior Blalock-Taussig shunt and pulmonary artery (PA) band. Total cardiopulmonary bypass (CPB) support times, circulatory arrest times, cross-clamp times, revision of the coronary anastomosis and delayed sternal closure were recorded. Postoperative data included length of mechanical support and ventilation, cardiac intensive care unit (ICU) and hospital lengths of stay, the need for early reoperation and mortality. In this study, early mortality was defined as death before hospital discharge or within 30 days of ASO. This, therefore, included one child who was discharged from the hospital on day 18 after surgery, and died at home five days later from a presumed arrhythmia, and one child who died 79 days postoperatively before discharge.
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| Results |
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Analysis of factors associated with early mortality. There were 16 early deaths (7%) in this cohort. Of the 109 patients who, according to criteria published in previous reports (10,14,15), would be considered "low risk" candidates for the ASO (i.e., full-term infants with d-TGA and intact ventricular septum repaired in the first 2 weeks of life), there were three early deaths (2.7%). The first patient was a 4.4-kg full-term infant with an intramural right coronary artery who had an Aubert-type coronary conduit (30) and died on the 8th postoperative day of myocardial ischemia. Patient 2 (with "usual" coronary anatomy) had ongoing left ventricular anterior wall ischemia postoperatively requiring a second period of CPB and a revision of the coronary anastomosis. He died six days postoperatively with multisystem organ failure from persistent low cardiac output. Patient 3 had a massive capillary leak syndrome and severe pulmonary edema immediately postoperatively, a large right ventricular thrombus requiring reoperation for thrombus removal, subsequent severe right ventricular failure leading to extracorporeal membrane oxygenation and multisystem organ failure. He died on postoperative day 9. When mortality data for the entire cohort are examined with respect to time after ASO, the probabilities of survival at 1 week, 1 month and 6 months are 97%, 94% and 92%, respectively (Fig. 2). The median time from surgery to early death was 9 days (range: 0 to 79 days).
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Using risk factors associated with early mortality after ASO in univariate analysis, two multivariate models were constructed (Table 5). The first model employed only those variables available to clinicians preoperatively (preoperative model). Stated differently, this model assesses the risk of mortality before performing the ASO. The second model employs both preoperative and intraoperative variables and aims to assess independent risk factors at the conclusion of surgery before arrival in the cardiac ICU (postoperative model). The preoperative model indicates that lower birth weight (odds ratio 2.6, p = 0.02) and aortic arch repair before ASO (odds ratio 12.3, p = 0.007) are both risk factors for early mortality. The odds ratio for birth weight is that associated with a 1-kg decrease in weight. The risk of early mortality in patients with a birthweight of
2.8 kg was significantly increased compared with those with a birthweight >2.8 kg (17% vs. 4%, p = 0.004). Pulmonary artery banding before ASO was also strongly associated with early mortality. However, its strong association with aortic arch repair before ASO made it difficult to separate the individual effects of these variables and precluded the inclusion of both in the same model.
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| Discussion |
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Early mortality and coronary pattern. Although previous studies found that complex coronary artery patterns were associated with increased mortality (1015,31), the results of this study show that the impact of coronary artery anatomy has diminished with surgical experience. Wernovsky et al. (12) found that inverted origins of the right and circumflex coronary arteries as well as the pattern of a single origin of the right and left anterior descending coronary arteries were risk factors for early mortality. Yamaguchi et al. (13) found an overall higher mortality rate in patients with unusual coronary anatomy. Mayer et al. (21) found that coronary anatomy impact over time seemed to decrease, despite an overall correlation of inverted coronary patterns with increased mortality rates. Similar to the results of this study, Quaegebeur and colleagues (1) found no effect of coronary anatomy pattern on early death in an early series of 66 patients. Although this study shows that coronary artery pattern was no longer associated with an increased risk of early mortality, there is an impact of coronary pattern on intraoperative variables and postoperative morbidity. Patients with inverted coronary origins and those with a single right coronary artery pattern had longer duration of cardiopulmonary bypass, a higher incidence of reintervention at the coronary anastomosis site and an increased risk of delayed sternal closure. Postoperatively, patients with inverted coronary anatomy had a longer duration of mechanical ventilation and a trend toward longer postoperative hospital stay. These findings likely reflect an evolution in progress of surgical management of complex coronary patterns. Although the techniques used to transfer these coronary arteries have evolved to such a degree that they are not associated with excess mortality, the time required to perform these procedures is still longer compared with uncomplicated coronary patterns.
Risk factors for early mortality. Lower birth weight infants continue to be at increased risk for both mortality and morbidity after cardiopulmonary bypass and complex congenital cardiac surgery. These data corroborate prior studies (10,12,19) showing that repair of aortic arch obstruction before ASO both with and without right ventricular hypoplasia are important independent risk factors for death in this population. Further investigation of the right ventricular structure is needed to more accurately evaluate the ability of a small right side to accommodate an ASO in these patients.
Because of the strong association of pulmonary artery band with early aortic arch repair, it was not possible to evaluate the independent effects of these covariables. Intraoperative circulatory arrest times as well as overall cardiopulmonary bypass times also continue to be independent risk factors for early mortality.
Timing of aortic arch repair. The surgical approach in patients with d-TGA or DORV and associated aortic arch anomalies is still under debate. Some centers have advocated a staged approach, with early repair of the aortic arch (either coarctation or interrupted arch repair) with or without placement of a PA band to restrict pulmonary blood flow, followed by ASO and PA-plasty at a later date. Although this approach affords a low initial mortality and a left thoracotomy access for the aortic arch, the potential disadvantages include branch pulmonary artery stenosis secondary to PA band manipulation, neoaortic valve insufficiency and adhesions as a result of prior surgical intervention. Other studies have advocated a single-stage repair for this lesion (3235). Planche et al. (35) reported a mortality rate of 14% with primary repair compared with 30.7% mortality with two-stage repair. These results, however, are difficult to interpret, since the two series were from different time periods. Several centers have reported the feasibility and success of the anterior approach for aortic arch repair (36,37). The data from this cohort suggest that the risks of repair of the aortic arch as a separate operation preceding the ASO exceeds the risk of the slightly longer intraoperative CPB times of the single-stage complete repair. The present study supports the feasibility of the single-stage approach, and more importantly, found that the two-stage approach for repair of aortic arch anomalies was an independent risk factor for early death. Single-stage repair with ASO and arch repair is now the procedure of choice for most patients with d-TGA and arch obstruction or interruption in our institution.
Arterial switch operation for Taussig-Bing anomaly. The procedure of choice for patients with Taussig-Bing type DORV remains controversial. Mavroudis et al. (18) compared the results of ASO (n = 16) with those of the Kawashima intraventricular repair (n = 4) in patients with Taussig-Bing anatomy and side-by-side great vessels. Similar to our data, they found a mortality in the ASO population of approximately 7%. None of the four patients who underwent the Kawashima operation had died. There is also evidence that the ASO can also be used successfully for Taussig-Bing anomaly with anteriorposterior great vessel anatomy (4,12,1720). The present study found that although the age at operation for this group was significantly older, the morbidity and mortality outcomes did not differ from the group of patients with d-TGA and VSD. The ASO continues to be the procedure of choice at this institution for patients with Taussig-Bing type DORV in which a biventricular repair can be performed.
Study limitations. Because the number of patients with intramural coronary arteries was small (n = 4), there was limited power for risk analysis of intramural coronary patterns. Another potential concern is the ability of the sample size in this cohort to detect an increase in risk of mortality secondary to abnormal coronary patterns. However, analysis of outcome data shows that the mortality rate in patients with usual coronary pattern and origin of the circumflex coronary artery from the right coronary artery (types I and II, Fig. 1) was 7.18%, and the mortality rate in patients with unusual patterns was 7.14%. Therefore, any difference in risk appears to be negligible. In addition to that limitation, comparison to the previous cohort, even within the same institution, is complicated by differing patient characteristics, shifting referral patterns and changing management strategies. The limitation of highly associated variables is inherent to our study population.
Conclusions. Results of this study indicate that the arterial switch operation continues to offer an excellent surgical option for infants with d-TGA and certain types of DORV, even in the presence of complex associated lesions. Complex coronary branching patterns were no longer associated with an increased risk of early death but continue to be associated with longer ICU and hospital stays. Lower birth weight, right ventricular hypoplasia and staged repair of aortic arch obstruction continue to be independent risk factors for mortality after the ASO. Ongoing assessment of risk factors and long-term follow-up of the outcome of these children are imperative for continued evaluation of the ASO.
| Acknowledgments |
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| Footnotes |
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| References |
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