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The influence of coronary artery anatomy on mortality after the arterial switch operation
CONGENITAL: TRANSPOSITION
The influence of coronary artery anatomy on mortality after the arterial switch operation
ABSTRACT Objective: We sought to determine the influence of coronary artery anatomy on mortality in more than 1000 children undergoing the arterial switch operation. Methods: All patients who underwent an arterial switch operation were identified from 2 hospital databases and reviewed retrospectively. Coronary anatomy was recorded from operative reports using the Leiden classification. Results: An arterial switch operation was performed in 1033 children between 1983 and 2013. Coronary anatomy was normal in 697 patients (67%). The most common type of anomalous coronary anatomy was the circumflex coronary artery arising from sinus 2 (in 152 patients [15%]). Forty-seven patients (4.5%) had all coronary arteries arising from a single sinus. Of these 47 patients, 34 patients (3.3%) had a true single coronary artery. Fifty-two patients (5.0%) had an intramural coronary artery. Overall early mortality was 3.3% (34 out of 1033 patients) over the 30-year period. Early mortality was 3.0% (21 out of 697) for patients with normal coronary anatomy and 3.9% (13 out of 336) for any type of anomalous coronary anatomy. Early mortality was 3.3% (5 out of 152) for patients with the circumflex coronary artery arising from sinus 2, 6.4% (3 out of 47) for patients with all coronary arteries arising from a single sinus, and 5.9% (2 out of 34) for patients with a true single coronary artery. Early mortality for patients with intramural coronaries was 1.9% (1 out of 52). No coronary pattern was found to be a risk factor for mortality. Conclusions: Patients with anomalous coronary artery anatomy had higher rates of early mortality after the arterial switch operation but this was not statistically significant. Coronary artery reoperations were rare. (J Thorac Cardiovasc Surg 2020;160:191-9)
• Low early mortality • Low rate of coronary reoperation
• Higher early mortality but not statistically significant • Higher rates of postoperative MCS • Low rate of coronary reoperation
Outcomes in patients with normal and abnormal coronary anatomy undergoing arterial switch.
CENTRAL MESSAGE
Patients with anomalous coronary anatomy had higher rates of early mortality after the ASO but this was not statistically significant and coronary reoperation was rare. PERSPECTIVE Patients with anomalous coronary anatomy undergoing the arterial switch operation are a challenging subgroup. These patients may be at a higher risk of mortality as well as a higher risk for postoperative mechanical circulatory support and coronary reintervention. See Commentaries on pages 200 and 201.
The arterial switch operation (ASO) has excellent early and late outcomes in the modern era.1-5 Technical complexity of the operation may increase in the presence of coronary artery anomalies, which occur in
approximately one-third of patients.1,2,6-8 In particular, single coronary arteries and intramural coronary arteries have been associated with higher mortality.9-11 Therefore, we sought to determine the influence of
From the aDepartment of Cardiac Surgery, The Royal Children’s Hospital, Melbourne, Australia; bUniversity of Melbourne, Melbourne, Australia; cMurdoch Children’s Research Institute, Melbourne, Australia; dDepartment of Cardiac Surgery, Queensland Children’s Hospital, Brisbane, Australia; and eMelbourne Children’s Centre for Cardiovascular Genomics and Regenerative Medicine, Melbourne, Australia. Supported by the Victorian Government’s Operational Infrastructure Support Program in Australia. Dr Fricke is a recipient of a postgraduate scholarship from the Australian National Health and Medical Research Council (No. 1134203). Dr d’Udekem is an Australian National Health and Medical Research Council Clinician Practitioner Fellow (No. 1082186).
Read at the 99th Annual Meeting of The American Association for Thoracic Surgery, Toronto, Ontario, Canada, May 4-7, 2019. Received for publication May 8, 2019; revisions received Nov 24, 2019; accepted for publication Nov 26, 2019; available ahead of print Feb 24, 2020. Address for reprints: Igor E. Konstantinov, MD, PhD, FRACS, Royal Children’s Hospital, Flemington Rd, Parkville, 3052, Australia (E-mail: igor.konstantinov@rch. org.au). 0022-5223/$36.00 Crown Copyright Ó 2020 Published by Elsevier Inc. on behalf of The American Association for Thoracic Surgery https://doi.org/10.1016/j.jtcvs.2019.11.146
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Tyson A. Fricke, MBBS, BMedSci,a,b,c Douglas Bell, MBBS,d Michael Daley, MBBS,a,d Yves d’Udekem, MD, PhD, FRACS,a,b,c Christian P. Brizard, MD,a,b,c Nelson Alphonso, MBBS, FRACS,d and Igor E. Konstantinov, MD, PhD, FRACSa,b,c,e
Congenital: Transposition
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Abbreviations and Acronyms ASO ¼ arterial switch operation Cx ¼ circumflex ECMO ¼ extracorporeal membrane oxygenation MCS ¼ mechanical circulatory support LAD ¼ left anterior descending NYHA ¼ New York Heart Association RCA ¼ right coronary artery TGA ¼ transposition of the great arteries TGA-IVS ¼ transposition of the great arteries with an intact ventricular septum without aortic arch obstruction TGA-VSD ¼ transposition of the great arteries with a ventricular septal defect without aortic arch obstruction
Scanning this QR code will take you to the article title page to access supplementary information. To view the AATS Annual Meeting Webcast, see the URL next to the webcast thumbnail. coronary artery anatomy on mortality in more than 1000 children undergoing ASO. MATERIALS AND METHODS Patients This study was approved by The Royal Children’s Hospital Human Research Ethics Committee and The Children’s Health Queensland Human Research Ethics Committee. All data were collected retrospectively from hospital records for patients operated at The Royal Children’s and Queensland Children’s Hospitals between 1983 and 2013. Data for patients who were aged 18 years or older were collected from hospital records of adult congenital cardiac services and general practitioners. Coronary anatomy was recorded from operative reports using the Leiden classification.
Definitions Early death or reoperation was defined as death or reoperation occurring before hospital discharge or within 30 days of ASO. Late death was defined as death occurring after discharge and more than 30 days after ASO. Coronary reintervention was defined as reoperation or transcatheter procedure on the coronary arteries. Normal coronary anatomy was defined as the left anterior descending artery (LAD) and circumflex coronary arteries (Cx) arising from sinus 1 and the right coronary artery (RCA) arising from sinus 2. All patterns other than this were considered abnormal or anomalous.
Statistical Analysis Data were imported into Stata version 12 (Stata Corp, College Station, Tex). Continuous variables were reported as a median with an interquartile range (IQR). Kaplan-Meier curves were constructed to display freedom from the study’s outcomes. Cox regression analysis was used to determine risk factors for statistical significance. Fisher exact test was used to detect significant differences in early mortality between groups. The Mann-
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Whitney U test was used to detect any significant differences in medians of continuous variables between groups.
RESULTS An ASO was performed in 1033 children between 1983 and 2013 at The Royal Children’s and Queensland Children’s Hospitals. The trapdoor technique for coronary translocation had been used at both institutions consistently since the inception of their programs in almost all patients. Table 1 summarizes the perioperative and operative data of the patients. Eight hundred eight patients (78%) underwent ASO at The Royal Children’s Hospital between 1983 and 2013. Two hundred twenty-five patients (22%) underwent ASO at the Queensland Children’s Hospital between 1995 and 2013. Nine hundred fifty (92%) had transposition of the great arteries (TGA), 67 patients (6.5%) had TaussigBing anomaly, and 16 patients (15%) had a previous ASO with takedown and conversion to ASO. In the patients with TGA, 282 (30%) had a ventricular septal defect (VSD) without aortic arch obstruction, 613 out of 950 patients (65%) had an intact interventricular septum (IVS) without aortic arch obstruction, and 55 out of 1033 patients (5.3%) had TGA with aortic arch obstruction. Median age of operation was 10 days (IQR, 7-20 days) and median weight at operation was 3.5 kg (IQR, 3.1-3.9 kg). Thirty-six patients (3.5%) weighed <2.5 kg at time of operation. Anomalous coronary anatomy occurred in 336 patients (33%). Anomalous coronary anatomy was more common in patients with VSD compared with those with an intact IVS (37% vs 30%; P ¼ .03). The most common type of anomalous coronary anatomy was the Cx arising from sinus 2 (15%). Forty-seven patients (4.5%) had all coronary arteries arising from a single sinus. Of these 47 patients, 34 (3.3%) had a true single coronary artery. Fifty-two patients (5.9%) had an intramural coronary artery. Follow-up after hospital discharge was 92% (947 patients). Median follow-up was 14.3 years (IQR, 8.3-18.8 y). Mortality There were 34 early deaths (3.3%). Table 2 lists the early mortality for each coronary pattern. Early mortality was 3.0% (21 out of 697) for patients with normal coronary anatomy and 3.9% (13 out of 336) for patients with anomalous coronary anatomy. Early mortality was 3.3% (5 out of 152) for patients with the Cx arising from sinus 2, 6.4% (3 out of 47) for patients with all coronary arteries arising from a single sinus, and 5.9% (2 out of 34) for patients with a true single coronary artery. Early mortality for patients with intramural coronaries was 1.9% (1 out of 52). There were no significant differences in early mortality among patients with normal coronary anatomy compared with anomalous coronary anatomy by decade operated (Table 3). Causes of death were coronary ischemia (n ¼ 22), sepsis (n ¼ 7), respiratory failure (n ¼ 2), unknown cause (n ¼ 2), and
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TABLE 1. Perioperative and operative data Anomalous coronary arteries (n ¼ 336)
P value
TGA
645 (93)
305 (91)
TGA-IVS
430 (62)
183 (54)
NS .03
TGA-VSD
178 (26)
104 (31)
NS
TGA-AAO
37 (5.3)
18 (5.4)
NS
TBA
37 (5.3)
30 (8.9)
.03
TBA without AAO
23 (3.3)
15 (4.5)
.04
TBA AAO
14 (2.0)
15 (4.5)
.04
Atrial switch to ASO conversion
15 (2.2)
1 (0.3)
.03
Median age at operation (d)
10 (7-19)
10 (7-22)
Median operative weight (kg)
3.1 (3.1-3.9)
3.5 (3.2-3.9)
NS
–
Operative weight <2.5 kg
24 (3.4)
12 (3.6)
NS –
Coronaries from single sinus
–
47 (14)
Coronaries from single sinus, single ostium*
–
34 (10)
–
Coronaries from single sinus but separate ostia
–
13 (3.9)
–
1LAD; 2RCA; Cx
–
152 (45)
–
1LAD, RCA; 2Cx
–
35 (10)
–
1RCA; 2LAD; Cx
–
24 (7.1)
–
1Cx; 2LAD, RCA
–
2 (0.6)
–
Intramural coronary artery
–
52 (15)
–
1 Conal branch; 2LAD, Cx, RCA
–
16 (4.8)
–
1 Accessory right ventricle branch; 2LAD, Cx, RCA
–
2 (0.6)
–
1LAD, Cx, RCA; 2 sinoatrial node branch
–
2 (0.6)
–
1 Accessory LAD, 2LAD, Cx, RCA
–
2 (0.6)
–
1LAD, Cx, RCA; 2 accessory RCA
–
1 (0.6)
–
1LAD, Cx; 2RCA, accessory LAD
–
Median CPB time (min)
1 (0.6)
–
164 (137-198)
175 (134-214)
NS
Median aortic crossclamp time (min)
88 (71-112)
97 (76-120)
NS
Postoperative MCS
20 (2.9)
22 (6.5)
<.001
Values are presented as n (%) or median (interquartile range). TGA, Transposition of the great arteries, NS, nonsignificant; IVS, intact interventricular septum; VSD, ventricular septal defect without aortic arch obstruction; AAO, aortic arch obstruction; TBA, Taussig-Bing anomaly; ASO, arterial switch operation, LAD, left anterior descending coronary artery, RCA, right coronary artery; Cx, circumflex coronary artery; CPB, cardiopulmonary bypass; MCS, mechanical circulatory support. *True single coronary artery.
brain death (n ¼ 1). Late mortality occurred in 9 out of 999 patients (0.9%), 2 of whom had an anomalous coronary anatomy. There was no statistically significant difference in early mortality between normal coronary anatomy and abnormal coronary anatomy in patients with TGA-IVS (1.2% vs 2.7%; P ¼ .17), patients with TGA-VSD (3.9% vs 5.8%; P ¼ .56), or overall (3.0% vs 3.9%; P ¼ .46). No coronary pattern was found to be a risk factor for mortality on Cox regression analyzed for the entire cohort, for TGA-IVS patients alone, and for TGA-VSD patients alone.
Survival at 20 years was 96% for both normal and abnormal coronary artery anatomy patients (Figure 1 and Table E1). In patients for whom New York Heart Association (NYHA) functional class was available at last follow-up, 98% of patients (587 out of 598) with normal coronary anatomy were in NYHA functional class I and 98% of patients (277 out 283) with an abnormal coronary anatomy were in NYHA functional class I. Postoperative mechanical circulatory support (MCS) was more common in patients with abnormal coronary artery anatomy compared with those with normal
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Normal coronary arteries (n ¼ 697)
Variable
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TABLE 2. Early mortality
Overall
TGA-IVS
Early mortality TBA without TGA-VSD TGA-AAO AAO TBA-AAO
1LAD, Cx; 2RCA
3.0 (21/697)
1.2 (5/430)
3.9 (7/178)
11 (4/37)
13 (3/23)
0 (0/14)
13 (2/15)
Abnormal coronary anatomy
3.9 (13/336)
2.7 (5/183)
5.8 (6/104)
11 (2/18)
0 (0/15)
0 (0/15)
0 (0/1)
Coronaries from single sinus
6.4 (3/47)
4.5 (1/22)
11 (2/18)
0 (0/5)
0 (0/2)
0 (0/0)
0 (0/0)
Coronaries from single sinus, single ostium*
8.3 (1/12)
0 (0/3)
0 (0/2)
0 (0/0)
0 (0/0)
0 (0/4)
0 (0/1)
0 (0/1)
0 (0/0)
0 (0/0)
14 (1/7)
0 (0/2)
0 (0/1)
0 (0/0)
0 (0/0)
Coronary pattern
Atrial switch to ASO conversion
CONG
5.9 (2/34)
5.9 (1/17)
True single CA from sinus 1
0 (0/16)
0 (0/10)
True single CA from sinus 2
13 (2/16)
17 (1/6)
True single CA from nonfacing sinus
50 (1/2)
100 (1/1)
0 (0/1)
0 (0/0)
0 (0/0)
0 (0/0)
0 (0/0)
Coronaries from single sinus, separate ostia
7.7 (1/13)
0 (0/5)
17 (1/6)
0 (0/2)
0 (0/0)
0 (0/0)
0 (0/0)
1LAD; 2RCA, Cx
3.3 (5/152)
2.4 (2/85)
2.1 (1/47)
29 (2/7)
0 (0/6)
0 (0/6)
0 (0/1)
1LAD, RCA; 2 Cx
8.3 (3/36)
11 (2/19)
10 (1/10)
0 (0/1)
0 (0/3)
0 (0/3)
0 (0/0)
1RCA; 2LAD, Cx
4.3 (1/23)
0 (0/6)
11 (1/9)
0 (0/1)
0 (0/4)
0 (0/3)
0 (0/0)
1Cx; 2LAD, RCA Intramural CA 1 Conal branch; 2LAD, Cx, RCA
0 (0/2)
0 (0/1)
0 (0/1)
0 (0/0)
0 (0/0)
0 (0/0)
0 (0/0)
1.9 (1/52)
0 (0/37)
11 (1/11)
0 (0/3)
0 (0/0)
0 (0/1)
0 (0/0)
0 (0/9)
0 (0/6)
0 (0/0)
0 (0/0)
0 (0/1)
0 (0/0)
0 (0/16)
1 Accessory RV branch; 2LAD, Cx, RCA
0 (0/2)
0 (0/0)
0 (0/1)
0 (0/1)
0 (0/0)
0 (0/0)
0 (0/0)
1LAD, Cx, RCA; 2 sinoatrial node branch
0 (0/2)
0 (0/1)
0 (0/1)
0 (0/0)
0 (0/0)
0 (0/0)
0 (0/0)
1 Accessory LAD, 2LAD, Cx, RCA
0 (0/2)
0 (0/2)
0 (0/0)
0 (0/0)
0 (0/0)
0 (0/0)
0 (0/0)
1LAD, Cx, RCA; 2 accessory RCA
0 (0/1)
0 (0/0)
0 (0/0)
0 (0/0)
0 (0/0)
0 (0/1)
0 (0/0)
1LAD, Cx; 2 RCA, accessory LAD
0 (0/1)
0 (0/1)
0 (0/0)
0 (0/0)
0 (0/0)
0 (0/0)
0 (0/0)
Values are presented as % (n/N). TGA, Transposition of the great arteries with aortic arch obstruction; IVS, intact interventricular septum without aortic arch obstruction; VSD, ventricular septal defect and no aortic arch obstruction; AAO, aortic arch obstruction; TBA, Taussig-Bing anomaly; ASO, aortic switch operation; LAD, left anterior descending artery; Cx, circumflex coronary artery; RCA, right coronary artery; CA, coronary artery; RV, right ventricular. *True single coronary artery.
coronary anatomy (6.5% vs 2.9%; P ¼ .007) as well as in patients with intramural coronary arteries (9.6%; P ¼ .02). Patients who required MCS had longer median cardiopulmonary bypass (235 minutes vs 109 minutes; P < .001) and longer median aortic crossclamp times (170 minutes vs 92 minutes; P < .001). Patients who required MCS had more associated VSD repairs (48% vs 39%; P ¼ .27) and more associated aortic arch obstruction repairs (17% vs 7.9%; P ¼ .07). More patients who required MCS weighed <2.5 kg at time of ASO (9.5% vs 3.2%; P ¼ .05). Reintervention for Coronary Stenosis Six patients (0.6%) required 8 reinterventions—all reoperations—on the coronary arteries at a median 11 days (IQR, 6-33 days; range, 1 day to 9 years) after ASO. Three patients with normal coronary anatomy (0.4%; 3 out of 697) required reintervention for coronary stenosis and 3 patients (0.9%; 3 out of 336) with abnormal coronary anatomy. The difference was not statistically significant (P ¼ .40).
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Patient 1 underwent an uneventful ASO for TGA-IVS at 8 days of age with LAD, RCA, and Cx coronary anatomy of 1LAD, 2RCA; Cx, and end-to-end coarctation repair at age 11 days. The patient had a very small, doubly committed VSD that was missed during the ASO. The patient developed chest pain 9 years after ASO and transthoracic echocardiography demonstrated partial occlusion of the LAD by the distorted cusp of the aortic valve. Aortic valve repair was performed, the VSD was closed and LAD was reimplanted. The patient is in NYHA functional class I at age 17 years. Patient 2 underwent an ASO for TGA-IVS at age 8 days with normal coronary anatomy. The patient’s immediate postoperative period was complicated by bradycardia and blood pressure dependent electrocardiogram changes. A coronary angiogram was performed demonstrating patent coronaries; however, there was concern for ongoing coronary ischemia. The patient returned to theatre and underwent relocation of the RCA button. The patient is in NYHA functional class I at age 13 years.
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TABLE 3. Early mortality by decade Era
Anatomy
Early mortality
1983-1989
Overall Normal CA anatomy Abnormal CA anatomy
3.4 (5/148) 4.9 (4/82) 1.5 (1/66)
Overall Normal CA anatomy Abnormal CA anatomy
3.4 (12/352) 3.5 (9/254) 3.1 (3/98)
Overall Normal CA anatomy Abnormal CA anatomy
3.2 (17/533) 2.2 (8/361) 3.1 (9/272)
1990-1999
2000-2013
Values are presented as % (n/N). CA, Coronary artery.
Patient 3 underwent ASO for TGA-IVS at age 18 days with coronary anatomy of 1LAD, RCA; 2Cx. Immediately after coming of bypass, there was ischemia of the posterior ventricular wall. The Cx button was repositioned and the child was supported with extracorporeal membrane oxygenation (ECMO). Unfortunately, the patient had cerebral embolism while on ECMO and died on day 12 after ASO. Patient 4 underwent ASO and end to side arch repair for Taussig-Bing anomaly with coarctation at age 6 days with
normal coronary anatomy. The patient had a cardiac arrest in the intensive care unit and was placed on ECMO. The coronary angiogram demonstrated severe stenosis of the left coronary artery and the left coronary button was relocated lower into the sinus of Valsalva. The patient is alive and well at age 7 years. Patient 5 underwent ASO for TGA-VSD at age 11 days with normal coronary anatomy. The patient went into ventricular tachycardia the day after ASO. Because of concern for right coronary ischemia, the patient returned to operating theatre for reimplantation of the RCA button. The patient is in NYHA functional class I at age 16 years. Patient 6 underwent ASO for TGA-IVS at 11 days of age with coronary anatomy of 1LAD, RCA; 2Cx. The patient was unable to be successfully weaned from cardiopulmonary bypass at time of ASO and was placed on ECMO. A coronary angiogram demonstrated obstruction at the level of the Cx and this was revised with a patch on day 9 postoperatively. Repeat coronary angiography demonstrated residual obstruction at the level of the Cx patch and the coronary was relocated to the nonfacing sinus. The patient was unable to be weaned off ECMO and died at day 23 postoperatively. DISCUSSION An anomalous coronary anatomy may represent a technical challenge during ASO. The most common abnormal anatomy is Cx coronary artery arising from sinus 2.1-3,6,7
Survival (%)
100
75
50
Number at risk Normal CA Abnormal CA Single CA Intramural CA
0
5
10 Years since ASO
15
20
697 250 34 52
544 189 23 43
416 148 19 38
293 102 14 26
106 53 10 14
FIGURE 1. Survival by coronary artery anatomy. Survival for patients with normal coronary arteries (CA) was 96% (95% confidence interval [95% CI], 94%-97%) at 5, 10, and 20 years after arterial switch operation (ASO). Survival for patients with CAs was 96% (95% CI, 92%-98%) at 5, 10, and 20 years after ASO. Survival for patients with single CA was 91% (95% CI, 74%-97%) at 5, 10, and 20 years after ASO. Survival for patients with intramural CAs was 98% (95% CI, 87%-100%) at 5, 10, and 20 years after ASO.
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ASO was performed in 1033 children from 1983–2013
Medical records were retrospectively reviewed
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• Low early mortality • Low rate of coronary reoperation
• Higher early mortality but not statistically significant • Higher rates of postoperative MCS • Low rate of coronary reoperation
FIGURE 2. Normal and abnormal coronary artery outcomes. Abnormal coronary artery anatomy associated with a higher early mortality but was not statistically significant and was still had a low rate of coronary reoperation. ASO, Arterial switch operation; RCA, right coronary artery; LAD, left anterior descending artery; LCx, left circumflex artery; MCS, mechanical circulatory support.
A single coronary artery—or all coronaries arising from the same sinus—are usually the next most common anomaly.1,3, Single coronary artery and intramural coronary arteries are uncommon coronary patterns and, thus, a large cohort of patients is necessary to analyze risk factors for mortality. Technical experience with complex coronary anomalies has improved the results of many institutions over the years. Despite this improvement, contradictory results still exist in the literature. The meta-analysis by Pasquali and colleagues10 on the effect of coronary anatomy on mortality after ASO has been referenced by many.6,7,9,11-16 In this metaanalysis of 1942 patients, any variant of coronary artery anatomy doubled the risk of death. Furthermore, the same meta-analysis demonstrated that risk of death in patients with a single coronary artery was 3 times higher and in those with an intramural coronary artery 7 times higher compared with those with normal coronary anatomy. This metaanalysis was limited to studies of patients undergoing operation between 1977 and 1999. A more recent study by Trezzi and colleagues6 investigated the effect of anomalous coronary anatomy in 283 children undergoing ASO between 2000 and 2015 and found it not to be a risk factor for operative mortality. Outcomes for intramural coronaries have been particularly varied with operative mortality ranging from 0% to 24% in recent studies.9,17,18 Likewise, single coronary arteries have also been a focus. The largest 196
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series of such patients comes from Gerelli and colleagues,11 who published results of 73 children with single coronary arteries undergoing ASO. Mortality was 11%. However, all deaths except 1 occurred in their patients operated before 2001. In our cohort of 1033 patients across 2 institutions, there were 2 (5.9%) operative deaths in patients with a single coronary artery and 1 (1.9%) operative death in patients with intramural coronaries. Both anomalies were not statistically significant risk factors for mortality. The technique for coronary translocation across both our institutions is the trapdoor technique, which has been previously described, and has been consistently used since its introduction by Mee and colleagues during the 1980s.2,18-20 We previously published a detailed description of our technique to translocate intramural coronary arteries.18 We observed higher early mortalities in TGA-IVS and TGA-VSD patients with abnormal coronary anatomy compared with normal coronary anatomy (1.2% vs 2.7% and 3.9% vs 5.8%) in the current study. Despite higher early mortality in children with anomalous coronary patterns, these differences between the groups were not statistically significant nor was anomalous coronary anatomy or any specific anomalous coronary pattern a risk factor on Cox regression analysis (Figure 2). We found that the need for postoperative MCS was more common in patients with abnormal coronary artery anatomy and intramural coronary arteries compared to those with normal coronary anatomy. However, this finding must be tempered by the fact that patients who required MCS had longer cardiopulmonary bypass and aortic crossclamp times, more concomitant VSD and aortic arch obstruction repairs, and were more likely to weigh<2.5 kg. Those factors, leading to more complex repairs and longer ischemic times, may be responsible for the higher need for MCS in these patients. Encouragingly, reinterventions for coronary stenosis were rare in patients (0.6%). Our 2 institutions do not perform routine coronary angiogram in patients after the ASO and do not have set protocols for coronary surveillance after ASO. However, most patients at both institutions undergo exercise stress testing before transfer to adult care. Stress testing that is suggestive of ischemia is followed with myocardial perfusion imaging or coronary angiography. Although no data exists on the ideal form of surveillance, it would be reasonable to routinely do an exercise test in asymptomatic patients with abnormal coronary artery anatomy on transition to adult congenital service. All patients with exercise-induced chest pain or those unable to complete exercise test should undergo computed tomography scan assessment of the coronary arteries. Coronary events are generally reported to be low late after ASO,1,3 but may be more common in institutions where routine follow-up coronary angiography is performed.2,21
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Limitations A limitation of this study was its retrospective nature. Differences in mortality may not be statistically significant due to small number of early deaths. Routine coronary angiography was not performed in our patients and therefore we cannot comment on the potential for coronary stenosis in asymptomatic patients. The rarity of certain types of anomalous coronaries as well as the low early mortality means significant differences in mortality are difficult to detect. CONCLUSIONS Anomalous coronary artery anatomy was not a statistically significant risk factor for mortality after ASO. However, patients with anomalous coronary anatomy had higher early mortality after ASO. Webcast You can watch a Webcast of this AATS meeting presentation by going to: https://aats.blob.core.windows.net/ media/19%20AM/Monday_May6/202BD/202BD/S90% 20-%20Coronary%20artery%20anomalies%20in%20 children/S90_5_webcast_053403526.mp4.
Conflict of Interest Statement Dr Brizard has a financial relationship with Admedus and Dr d’Udekem has financial relationships with Actelion and MSD. All other authors have nothing to disclose with regard to commercial support. References 1. Fricke TA, d’Udekem Y, Richardson M, Thuys C, Dronavalli M, Ramsay JM, et al. Outcomes of the arterial switch operation for transposition of the great arteries: 25 years of experience. Ann Thorac Surg. 2012;94:139-45. 2. Fricke TA, Konstantinov IE. Arterial switch operation: operative approach and outcomes. Ann Thorac Surg. 2019;107:302-10. 3. Khairy P, Clair M, Fernandes SM, Blume ED, Powell AJ, Newburger JW, et al. Cardiovascular outcomes after the arterial switch operation for D-transposition of the great arteries. Circulation. 2013;127:331-9. 4. Wong SH, Finucane K, Kerr AR, O’Donnell C, West T, Gentles TL. Cardiac outcome up to 15 years after the arterial switch operation. Heart Lung Circ. 2008;17:48-53. 5. Fricke TA, Loyer BR, Huang L, Griffiths S, Yaftian N, Dalziel KM, et al. Longterm quality of life in adult survivors after the arterial switch operation. Eur J Cardiothorac Surg. 2018;54:1001-3. 6. Trezzi M, Polito A, Albano A, Albanese SB, Cetrano E, Carotti A. Intraoperative coronary revision but not coronary pattern is associated with mortality after arterial switch operation. Eur J Cardiothorac Surg. 2017;52:83-9. 7. Moll M, Michalak KW, Sobczak-Budlewska K, Moll JA, Kopala M, Szymczyk K, et al. Coronary artery anomalies in patients with transposition of the great arteries and their impact on postoperative outcomes. Ann Thorac Surg. 2017;104:1620-8. 8. Lalezari S, Bruggemans EF, Blom NA, Hazekamp MG. Thirty-year experience with the arterial switch operation. Ann Thorac Surg. 2011;92: 973-9. 9. Metton O, Calvaruso D, Gaudin R, Mussa S, Raisky O, Bonnet D, et al. Intramural coronary arteries and outcome of neonatal arterial switch operation. Eur J Cardiothorac Surg. 2010;37:1246-53. 10. Pasquali SK, Hasselblad V, Li JS, Kong DF, Sanders SP. Coronary artery pattern and outcome of arterial switch operation for transposition of the great arteries: a meta-analysis. Circulation. 2002;106:2575-80. 11. Gerelli S, Pontailler M, Rochas B, Angeli E, Van Steenberghe M, Bonnet D, et al. Single coronary artery and neonatal arterial switch operation: early and longterm outcomes. Eur J Cardiothorac Surg. 2017;52:90-5. 12. Al Anani S, Fughhi I, Taqatqa A, Elzein C, Ilbawi MN, Polimenakos AC. Transposition of great arteries with complex coronary artery variants: time-related events following arterial switch operation. Pediatr Cardiol. 2017; 38:513-24. 13. Machida D, Isomatsu Y, Goda M, Suzuki S, Asou T, Masuda M. Successful coronary transfer for transposition of the great arteries with bilateral intramural coronary arteries from a single aortic sinus. Gen Thorac Cardiovasc Surg. 2018;66: 476-9. 14. Kitamura S. Pediatric coronary artery bypass surgery for congenital heart disease. Ann Thorac Surg. 2018;106:1570-7. 15. Ou P, Khraiche D, Celermajer DS, Agnoletti G, Le Quan Sang K-H, Thalabard JC, et al. Mechanisms of coronary complications after the arterial switch for transposition of the great arteries. J Thorac Cardiovasc Surg. 2013; 145:1263-9. 16. Mavroudis C, Stewart RD, Backer CL, Rudra H, Vargo P, Jacobs ML. Reoperative techniques for complications after arterial switch. Ann Thorac Surg. 2011; 92:1747-54. 17. Thrupp SF, Gentles TL, Kerr AR, Finucane K. Arterial switch operation: early and late outcome for intramural coronary arteries. Ann Thorac Surg. 2012;94: 2084-90. 18. Fricke TA, Bulstra AE, Naimo PS, Bullock A, Robertson T, d’Udekem Y, et al. Excellent long-term outcomes of the arterial switch operation in patients with intramural coronary arteries. Ann Thorac Surg. 2016;101:725-9. 19. Asou T, Karl TR, Pawade A, Mee RBB. Arterial switch-translocation of the intramural coronary artery. Ann Thorac Surg. 1994;57:461-5. 20. Fricke TA, Konstantinov IE. Translocation of intramural coronary artery at the arterial switch operation: divide and conquer? J Thorac Cardiovasc Surg. 2018;155:e131-2.
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Asymptomatic stenosis has been reported in approximately 10% of patients on coronary angiography or computed tomography coronary angiogram15,22 and abnormal coronary artery patterns have been identified as risk factors for coronary obstruction.21,23,24 When late coronary ischemia does occur, surgical coronary revascularization can be performed with a low mortality.16,25-28 The role of percutaneous coronary intervention in growing children is less well defined,14,29 but may play a larger role in adult patients in the future. Our results demonstrate that anomalous coronary arteries are not a risk factor for ASO. Reports of successful ASOs in even the most difficult coronary anatomy have been published, including a single coronary artery from the nonfacing sinus30 and bilateral intramural coronaries from a single sinus.13,31 Despite this, myocardial ischemia is the most common cause of early mortality and, although rare, late death is also coronary-related in many instances.32 Furthermore, the demonstration of early atherosclerosis by intravascular ultrasound in coronaries after ASO33 highlights the need for ongoing coronary surveillance throughout adulthood despite good outcomes.
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21. Legendre A, Losay J, Touchot-Kone A, Serraf A, Belli E, Piot JD, et al. Coronary events after arterial switch operation for transposition of the great arteries. Circulation. 2003;108:186-90. 22. Tsuda T, Bhat AM, Robinson BW, Baffa JM, Radtke W. Coronary artery problems late after arterial switch operation for transposition of the great arteries. Circ J. 2015;79:2372-9. 23. Angeli E, Formigari R, Pace Napoleone C, Oppido G, Ragni L, Picchio FM, et al. Long-term coronary artery outcome after arterial switch operation for transposition of the great arteries. Eur J Cardiothorac Surg. 2010;38: 714-20. 24. Bonhoeffer P, Bonnet D, Piechaud J-F, St€umper O, Aggoun Y, Villain E, et al. Coronary artery obstruction after the arterial switch operation for transposition of the great arteries in newborns. J Am Coll Cardiol. 1997;29: 202-6. 25. Angeli E, Raisky O, Bonnet D, Sidi D, Vouhe PR. Late reoperations after neonatal arterial switch operation for transposition of the great arteries. Eur J Cardiothorac Surg. 2008;34:32-6. 26. Raisky O, Bergoend E, Agnoletti G, Ou P, Bonnet D, Sidi D, et al. Late coronary artery lesions after neonatal arterial switch operation: results of surgical coronary revascularization. Eur J Cardiothorac Surg. 2007; 31:894-8. 27. Jung JC, Kwak JG, Kim ER, Bang JH, Min J, Lim JH, et al. Reoperation for coronary artery stenosis after arterial switch operation. Interact Cardiovasc Thorac Surg. 2018;27:169-76. 28. Mavroudis C, Backer CL, Duffy CE, Pahl E, Wax DF. Pediatric coronary artery bypass for Kawasaki congenital, post arterial switch, and iatrogenic lesions. Ann Thorac Surg. 1999;68:506-12. 29. Kampmann C, Kuroczynski W, Trubel H, Knuf M, Schneider M, Heinemann MK. Late results after PTCA for coronary stenosis after the arterial switch procedure for transposition of the great arteries. Ann Thorac Surg. 2005; 80:1641-6. 30. Konstantinov IE, Fricke TA, d’Udekem Y, Radford DJ. Translocation of a single coronary artery from the nonfacing sinus in the arterial switch operation: longterm patency of the interposition graft. J Thorac Cardiovasc Surg. 2010;140: 1193-4. 31. Cetrano E, Carotti A. Surgical treatment of transposition of the great arteries with bilateral intramural coronary arteries. Ann Thorac Surg. 2012; 93:986-7. 32. Losay J, Touchot A, Serraf A, Litvinova A, Lambert V, Piot JD, et al. Late outcome after arterial switch operation for transposition of the great arteries. Circulation. 2001;104:121-6. 33. Pedra SRFF, Pedra CAC, Abizaid AA, Braga SLN, Staico R, Arrieta R, et al. Intracoronary ultrasound assessment late after the arterial switch operation for transposition of the great arteries. J Am Coll Cardiol. 2005;45: 2061-8.
Key Words: arterial switch operation, anomalous coronary artery
Discussion Dr Jonathan M. Chen (Philadelphia, Pa). The group at Royal Children’s and Queensland Hospitals are truly to be commended on an enviable series of more than 1000 arterial switch operations with outstanding results, and certainly since the era of transesophageal echocardiography, congenital surgeons have come a long way in developing safe and reproducible techniques to overcome the challenges of complex coronary transfer. Single coronary, single ostium,
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double looping, intramural, all these variants had, for the most part, extraordinarily low mortality in this series across 30 years of study. It is really truly remarkable. My only comment would be that in deference to Jan Quaegebeur, there is no such thing as normal anatomy, there’s only usual anatomy. Six patients required surgical coronary reinterventions about 2 weeks after their switch. In retrospect, was there anything about the reinterventions you did that would inform what you should have done the first time around? Dr Tyson Alexander Fricke (Melbourne, Australia). I think the straight answer is no. In those 6 patients, 5 required early reoperation, and in those 5 patients, 3 had normal anatomy and they had what seemed like routine, normal arterial switch but experienced cardiac arrest during the early postop period, usually the first or second day. And on revision, in all 3 of those patients, there was kinking of the left coronary button. But nothing specific that I can identify looking through their original operations and the revision operations that would inform practice. Dr Chen. During the Van Arsdell-Calderone dynasty they suggested the intraoperative revision of coronary anastomoses based on echocardiogram findings in the operating room in terms of flow within the coronaries. Do you routinely do postswitch evaluation with transesophageal echocardiography and/or epicardial, and do you look at the coronary flow? Dr Fricke. To my knowledge, no, but perhaps Dr d’Udekem could elaborate on that. Dr Yves d’Udekem (Melbourne, Australia). We do not check the coronary blood flow at the end of an arterial switch systematically in all patients. Some surgeons in our unit do, some not. If we have the slightest issue, we check these flows with echocardiography and we have a very low threshold to reposition the coronary buttons if any doubt is present. Dr Chen. Fair enough. There are several reports that have been out there that suggest there is a surprisingly high rate of silent coronary occlusion late, like years and years and years after the procedure. Do you have any speculation about what we should think about going forward, what your program’s plans are to interrogate this about the long-term follow-up patients who have undergone arterial switch?
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Dr Fricke. At the Royal Children’s there is no standardized protocol for long-term coronary surveillance. Patients often follow-up in the hospital outpatient clinic, but they are also followed by individual cardiologists, so there is a bit of interclinician variability in their surveillance. Typically, patients will have an exercise stress test in early adolescence, and on transfer from pediatric to adult care, often there is a computed tomography coronary angiogram performed. But neither institution uses routine coronary angiograms. But looking, as you mentioned, in other articles published elsewhere, there are some instances of silent coronary obstruction, so perhaps that is something that needs to be re-evaluated. Dr Chen. Thanks. Great presentation. Dr d’Udekem. If you look at the recent literature, the incidence of silent coronary occlusion is relatively small, 5% at most. But the incidence of symptomatic coronary occlusion is even smaller. It remains difficult to know exactly how to organize the follow-up of these patients. Dr James Jaggers (Aurora, Colo). My question has to do with preoperative imaging. Sometimes we get an echocardiogram and 1 of the coronary arteries isn’t visible, and my reflex response is to get a computed tomography scan because I want to know what it looks like. If the outcomes are equivalent, does it really matter? Can you just go to the operating room and deal with it then? It sounds like if the outcomes are not really different, depending on anatomy, am I being unnecessarily concerned? Dr d’Udekem. Some of our cardiologists ask for a computed tomography scan, but it is variable among cardiologists. I have to say, I was trained in Great Ormond
Street Hospital at the time of Marc de Leval doing his human factors studies. For those who don’t know his study, they were looking at all the different parameters that could influence the outcomes and in particular the psychological factors. He found that if you have a wrong diagnosis of the coronary anatomy, that had an influence on your outcome. Whether the cardiologist identified that it was intramural and actually it was not intramural or if it’s an intramural and it was not expected, in both cases, it influenced the outcome. So, yes, one could wonder whether you should look too closely at the report of the cardiologist if you are not certain that it is adequate. Dr Joseph Forbess (Chicago, Ill). In the patients that you placed on mechanical circulatory support, and clearly that was higher in the abnormal coronaries, were any salvaged with transplant, and if so, were they counted among your survivors? How did you account for them? Dr Fricke. No patients proceeded to transplant. Dr Olivier Ghez. A very quick question. Some patients, of course, will occlude a coronary and be asymptomatic in the long term. Do you do checks if you had difficulties at reimplantation or anything? Do you do regular coronary imaging in cases of difficulty at reimplantation? Even if they do well, they may develop secondarily an obstruction even with being asymptomatic initially. Dr d’Udekem. It has been recommended to follow more closely those who had coronary abnormalities or any issues requiring reimplantation of a coronary artery and that may be the way to go.
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TABLE E1. Survival after arterial switch operation (ASO) by coronary pattern Coronary pattern
5y
10 y
20 y
Normal
96 (94-97)
96 (94-97)
96 (94-97)
Anomalous
96 (92-98)
96 (92-98)
96 (92-98)
Single coronary
91 (74-97)
91 (74-97)
91 (74-97)
Intramural coronary
98 (87-100)
98 (87-100)
98 (87-100)
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Values are presented as % (95% confidence interval).
199.e1
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The influence of coronary artery anatomy on mortality after the arterial switch operation
ABSTRACT Objective: We sought to determine the influence of coronary artery anatomy on mortality in more than 1000 children undergoing the arterial switch operation. Methods: All patients who underwent an arterial switch operation were identified from 2 hospital databases and reviewed retrospectively. Coronary anatomy was recorded from operative reports using the Leiden classification. Results: An arterial switch operation was performed in 1033 children between 1983 and 2013. Coronary anatomy was normal in 697 patients (67%). The most common type of anomalous coronary anatomy was the circumflex coronary artery arising from sinus 2 (in 152 patients [15%]). Forty-seven patients (4.5%) had all coronary arteries arising from a single sinus. Of these 47 patients, 34 patients (3.3%) had a true single coronary artery. Fifty-two patients (5.0%) had an intramural coronary artery. Overall early mortality was 3.3% (34 out of 1033 patients) over the 30-year period. Early mortality was 3.0% (21 out of 697) for patients with normal coronary anatomy and 3.9% (13 out of 336) for any type of anomalous coronary anatomy. Early mortality was 3.3% (5 out of 152) for patients with the circumflex coronary artery arising from sinus 2, 6.4% (3 out of 47) for patients with all coronary arteries arising from a single sinus, and 5.9% (2 out of 34) for patients with a true single coronary artery. Early mortality for patients with intramural coronaries was 1.9% (1 out of 52). No coronary pattern was found to be a risk factor for mortality. Conclusions: Patients with anomalous coronary artery anatomy had higher rates of early mortality after the arterial switch operation but this was not statistically significant. Coronary artery reoperations were rare. (J Thorac Cardiovasc Surg 2020;160:191-9)
• Low early mortality • Low rate of coronary reoperation
• Higher early mortality but not statistically significant • Higher rates of postoperative MCS • Low rate of coronary reoperation
Outcomes in patients with normal and abnormal coronary anatomy undergoing arterial switch.
CENTRAL MESSAGE
Patients with anomalous coronary anatomy had higher rates of early mortality after the ASO but this was not statistically significant and coronary reoperation was rare. PERSPECTIVE Patients with anomalous coronary anatomy undergoing the arterial switch operation are a challenging subgroup. These patients may be at a higher risk of mortality as well as a higher risk for postoperative mechanical circulatory support and coronary reintervention. See Commentaries on pages 200 and 201.
The arterial switch operation (ASO) has excellent early and late outcomes in the modern era.1-5 Technical complexity of the operation may increase in the presence of coronary artery anomalies, which occur in
approximately one-third of patients.1,2,6-8 In particular, single coronary arteries and intramural coronary arteries have been associated with higher mortality.9-11 Therefore, we sought to determine the influence of
From the aDepartment of Cardiac Surgery, The Royal Children’s Hospital, Melbourne, Australia; bUniversity of Melbourne, Melbourne, Australia; cMurdoch Children’s Research Institute, Melbourne, Australia; dDepartment of Cardiac Surgery, Queensland Children’s Hospital, Brisbane, Australia; and eMelbourne Children’s Centre for Cardiovascular Genomics and Regenerative Medicine, Melbourne, Australia. Supported by the Victorian Government’s Operational Infrastructure Support Program in Australia. Dr Fricke is a recipient of a postgraduate scholarship from the Australian National Health and Medical Research Council (No. 1134203). Dr d’Udekem is an Australian National Health and Medical Research Council Clinician Practitioner Fellow (No. 1082186).
Read at the 99th Annual Meeting of The American Association for Thoracic Surgery, Toronto, Ontario, Canada, May 4-7, 2019. Received for publication May 8, 2019; revisions received Nov 24, 2019; accepted for publication Nov 26, 2019; available ahead of print Feb 24, 2020. Address for reprints: Igor E. Konstantinov, MD, PhD, FRACS, Royal Children’s Hospital, Flemington Rd, Parkville, 3052, Australia (E-mail: igor.konstantinov@rch. org.au). 0022-5223/$36.00 Crown Copyright Ó 2020 Published by Elsevier Inc. on behalf of The American Association for Thoracic Surgery https://doi.org/10.1016/j.jtcvs.2019.11.146
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Tyson A. Fricke, MBBS, BMedSci,a,b,c Douglas Bell, MBBS,d Michael Daley, MBBS,a,d Yves d’Udekem, MD, PhD, FRACS,a,b,c Christian P. Brizard, MD,a,b,c Nelson Alphonso, MBBS, FRACS,d and Igor E. Konstantinov, MD, PhD, FRACSa,b,c,e
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Abbreviations and Acronyms ASO ¼ arterial switch operation Cx ¼ circumflex ECMO ¼ extracorporeal membrane oxygenation MCS ¼ mechanical circulatory support LAD ¼ left anterior descending NYHA ¼ New York Heart Association RCA ¼ right coronary artery TGA ¼ transposition of the great arteries TGA-IVS ¼ transposition of the great arteries with an intact ventricular septum without aortic arch obstruction TGA-VSD ¼ transposition of the great arteries with a ventricular septal defect without aortic arch obstruction
Scanning this QR code will take you to the article title page to access supplementary information. To view the AATS Annual Meeting Webcast, see the URL next to the webcast thumbnail. coronary artery anatomy on mortality in more than 1000 children undergoing ASO. MATERIALS AND METHODS Patients This study was approved by The Royal Children’s Hospital Human Research Ethics Committee and The Children’s Health Queensland Human Research Ethics Committee. All data were collected retrospectively from hospital records for patients operated at The Royal Children’s and Queensland Children’s Hospitals between 1983 and 2013. Data for patients who were aged 18 years or older were collected from hospital records of adult congenital cardiac services and general practitioners. Coronary anatomy was recorded from operative reports using the Leiden classification.
Definitions Early death or reoperation was defined as death or reoperation occurring before hospital discharge or within 30 days of ASO. Late death was defined as death occurring after discharge and more than 30 days after ASO. Coronary reintervention was defined as reoperation or transcatheter procedure on the coronary arteries. Normal coronary anatomy was defined as the left anterior descending artery (LAD) and circumflex coronary arteries (Cx) arising from sinus 1 and the right coronary artery (RCA) arising from sinus 2. All patterns other than this were considered abnormal or anomalous.
Statistical Analysis Data were imported into Stata version 12 (Stata Corp, College Station, Tex). Continuous variables were reported as a median with an interquartile range (IQR). Kaplan-Meier curves were constructed to display freedom from the study’s outcomes. Cox regression analysis was used to determine risk factors for statistical significance. Fisher exact test was used to detect significant differences in early mortality between groups. The Mann-
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Whitney U test was used to detect any significant differences in medians of continuous variables between groups.
RESULTS An ASO was performed in 1033 children between 1983 and 2013 at The Royal Children’s and Queensland Children’s Hospitals. The trapdoor technique for coronary translocation had been used at both institutions consistently since the inception of their programs in almost all patients. Table 1 summarizes the perioperative and operative data of the patients. Eight hundred eight patients (78%) underwent ASO at The Royal Children’s Hospital between 1983 and 2013. Two hundred twenty-five patients (22%) underwent ASO at the Queensland Children’s Hospital between 1995 and 2013. Nine hundred fifty (92%) had transposition of the great arteries (TGA), 67 patients (6.5%) had TaussigBing anomaly, and 16 patients (15%) had a previous ASO with takedown and conversion to ASO. In the patients with TGA, 282 (30%) had a ventricular septal defect (VSD) without aortic arch obstruction, 613 out of 950 patients (65%) had an intact interventricular septum (IVS) without aortic arch obstruction, and 55 out of 1033 patients (5.3%) had TGA with aortic arch obstruction. Median age of operation was 10 days (IQR, 7-20 days) and median weight at operation was 3.5 kg (IQR, 3.1-3.9 kg). Thirty-six patients (3.5%) weighed <2.5 kg at time of operation. Anomalous coronary anatomy occurred in 336 patients (33%). Anomalous coronary anatomy was more common in patients with VSD compared with those with an intact IVS (37% vs 30%; P ¼ .03). The most common type of anomalous coronary anatomy was the Cx arising from sinus 2 (15%). Forty-seven patients (4.5%) had all coronary arteries arising from a single sinus. Of these 47 patients, 34 (3.3%) had a true single coronary artery. Fifty-two patients (5.9%) had an intramural coronary artery. Follow-up after hospital discharge was 92% (947 patients). Median follow-up was 14.3 years (IQR, 8.3-18.8 y). Mortality There were 34 early deaths (3.3%). Table 2 lists the early mortality for each coronary pattern. Early mortality was 3.0% (21 out of 697) for patients with normal coronary anatomy and 3.9% (13 out of 336) for patients with anomalous coronary anatomy. Early mortality was 3.3% (5 out of 152) for patients with the Cx arising from sinus 2, 6.4% (3 out of 47) for patients with all coronary arteries arising from a single sinus, and 5.9% (2 out of 34) for patients with a true single coronary artery. Early mortality for patients with intramural coronaries was 1.9% (1 out of 52). There were no significant differences in early mortality among patients with normal coronary anatomy compared with anomalous coronary anatomy by decade operated (Table 3). Causes of death were coronary ischemia (n ¼ 22), sepsis (n ¼ 7), respiratory failure (n ¼ 2), unknown cause (n ¼ 2), and
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TABLE 1. Perioperative and operative data Anomalous coronary arteries (n ¼ 336)
P value
TGA
645 (93)
305 (91)
TGA-IVS
430 (62)
183 (54)
NS .03
TGA-VSD
178 (26)
104 (31)
NS
TGA-AAO
37 (5.3)
18 (5.4)
NS
TBA
37 (5.3)
30 (8.9)
.03
TBA without AAO
23 (3.3)
15 (4.5)
.04
TBA AAO
14 (2.0)
15 (4.5)
.04
Atrial switch to ASO conversion
15 (2.2)
1 (0.3)
.03
Median age at operation (d)
10 (7-19)
10 (7-22)
Median operative weight (kg)
3.1 (3.1-3.9)
3.5 (3.2-3.9)
NS
–
Operative weight <2.5 kg
24 (3.4)
12 (3.6)
NS –
Coronaries from single sinus
–
47 (14)
Coronaries from single sinus, single ostium*
–
34 (10)
–
Coronaries from single sinus but separate ostia
–
13 (3.9)
–
1LAD; 2RCA; Cx
–
152 (45)
–
1LAD, RCA; 2Cx
–
35 (10)
–
1RCA; 2LAD; Cx
–
24 (7.1)
–
1Cx; 2LAD, RCA
–
2 (0.6)
–
Intramural coronary artery
–
52 (15)
–
1 Conal branch; 2LAD, Cx, RCA
–
16 (4.8)
–
1 Accessory right ventricle branch; 2LAD, Cx, RCA
–
2 (0.6)
–
1LAD, Cx, RCA; 2 sinoatrial node branch
–
2 (0.6)
–
1 Accessory LAD, 2LAD, Cx, RCA
–
2 (0.6)
–
1LAD, Cx, RCA; 2 accessory RCA
–
1 (0.6)
–
1LAD, Cx; 2RCA, accessory LAD
–
Median CPB time (min)
1 (0.6)
–
164 (137-198)
175 (134-214)
NS
Median aortic crossclamp time (min)
88 (71-112)
97 (76-120)
NS
Postoperative MCS
20 (2.9)
22 (6.5)
<.001
Values are presented as n (%) or median (interquartile range). TGA, Transposition of the great arteries, NS, nonsignificant; IVS, intact interventricular septum; VSD, ventricular septal defect without aortic arch obstruction; AAO, aortic arch obstruction; TBA, Taussig-Bing anomaly; ASO, arterial switch operation, LAD, left anterior descending coronary artery, RCA, right coronary artery; Cx, circumflex coronary artery; CPB, cardiopulmonary bypass; MCS, mechanical circulatory support. *True single coronary artery.
brain death (n ¼ 1). Late mortality occurred in 9 out of 999 patients (0.9%), 2 of whom had an anomalous coronary anatomy. There was no statistically significant difference in early mortality between normal coronary anatomy and abnormal coronary anatomy in patients with TGA-IVS (1.2% vs 2.7%; P ¼ .17), patients with TGA-VSD (3.9% vs 5.8%; P ¼ .56), or overall (3.0% vs 3.9%; P ¼ .46). No coronary pattern was found to be a risk factor for mortality on Cox regression analyzed for the entire cohort, for TGA-IVS patients alone, and for TGA-VSD patients alone.
Survival at 20 years was 96% for both normal and abnormal coronary artery anatomy patients (Figure 1 and Table E1). In patients for whom New York Heart Association (NYHA) functional class was available at last follow-up, 98% of patients (587 out of 598) with normal coronary anatomy were in NYHA functional class I and 98% of patients (277 out 283) with an abnormal coronary anatomy were in NYHA functional class I. Postoperative mechanical circulatory support (MCS) was more common in patients with abnormal coronary artery anatomy compared with those with normal
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Normal coronary arteries (n ¼ 697)
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TABLE 2. Early mortality
Overall
TGA-IVS
Early mortality TBA without TGA-VSD TGA-AAO AAO TBA-AAO
1LAD, Cx; 2RCA
3.0 (21/697)
1.2 (5/430)
3.9 (7/178)
11 (4/37)
13 (3/23)
0 (0/14)
13 (2/15)
Abnormal coronary anatomy
3.9 (13/336)
2.7 (5/183)
5.8 (6/104)
11 (2/18)
0 (0/15)
0 (0/15)
0 (0/1)
Coronaries from single sinus
6.4 (3/47)
4.5 (1/22)
11 (2/18)
0 (0/5)
0 (0/2)
0 (0/0)
0 (0/0)
Coronaries from single sinus, single ostium*
8.3 (1/12)
0 (0/3)
0 (0/2)
0 (0/0)
0 (0/0)
0 (0/4)
0 (0/1)
0 (0/1)
0 (0/0)
0 (0/0)
14 (1/7)
0 (0/2)
0 (0/1)
0 (0/0)
0 (0/0)
Coronary pattern
Atrial switch to ASO conversion
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5.9 (2/34)
5.9 (1/17)
True single CA from sinus 1
0 (0/16)
0 (0/10)
True single CA from sinus 2
13 (2/16)
17 (1/6)
True single CA from nonfacing sinus
50 (1/2)
100 (1/1)
0 (0/1)
0 (0/0)
0 (0/0)
0 (0/0)
0 (0/0)
Coronaries from single sinus, separate ostia
7.7 (1/13)
0 (0/5)
17 (1/6)
0 (0/2)
0 (0/0)
0 (0/0)
0 (0/0)
1LAD; 2RCA, Cx
3.3 (5/152)
2.4 (2/85)
2.1 (1/47)
29 (2/7)
0 (0/6)
0 (0/6)
0 (0/1)
1LAD, RCA; 2 Cx
8.3 (3/36)
11 (2/19)
10 (1/10)
0 (0/1)
0 (0/3)
0 (0/3)
0 (0/0)
1RCA; 2LAD, Cx
4.3 (1/23)
0 (0/6)
11 (1/9)
0 (0/1)
0 (0/4)
0 (0/3)
0 (0/0)
1Cx; 2LAD, RCA Intramural CA 1 Conal branch; 2LAD, Cx, RCA
0 (0/2)
0 (0/1)
0 (0/1)
0 (0/0)
0 (0/0)
0 (0/0)
0 (0/0)
1.9 (1/52)
0 (0/37)
11 (1/11)
0 (0/3)
0 (0/0)
0 (0/1)
0 (0/0)
0 (0/9)
0 (0/6)
0 (0/0)
0 (0/0)
0 (0/1)
0 (0/0)
0 (0/16)
1 Accessory RV branch; 2LAD, Cx, RCA
0 (0/2)
0 (0/0)
0 (0/1)
0 (0/1)
0 (0/0)
0 (0/0)
0 (0/0)
1LAD, Cx, RCA; 2 sinoatrial node branch
0 (0/2)
0 (0/1)
0 (0/1)
0 (0/0)
0 (0/0)
0 (0/0)
0 (0/0)
1 Accessory LAD, 2LAD, Cx, RCA
0 (0/2)
0 (0/2)
0 (0/0)
0 (0/0)
0 (0/0)
0 (0/0)
0 (0/0)
1LAD, Cx, RCA; 2 accessory RCA
0 (0/1)
0 (0/0)
0 (0/0)
0 (0/0)
0 (0/0)
0 (0/1)
0 (0/0)
1LAD, Cx; 2 RCA, accessory LAD
0 (0/1)
0 (0/1)
0 (0/0)
0 (0/0)
0 (0/0)
0 (0/0)
0 (0/0)
Values are presented as % (n/N). TGA, Transposition of the great arteries with aortic arch obstruction; IVS, intact interventricular septum without aortic arch obstruction; VSD, ventricular septal defect and no aortic arch obstruction; AAO, aortic arch obstruction; TBA, Taussig-Bing anomaly; ASO, aortic switch operation; LAD, left anterior descending artery; Cx, circumflex coronary artery; RCA, right coronary artery; CA, coronary artery; RV, right ventricular. *True single coronary artery.
coronary anatomy (6.5% vs 2.9%; P ¼ .007) as well as in patients with intramural coronary arteries (9.6%; P ¼ .02). Patients who required MCS had longer median cardiopulmonary bypass (235 minutes vs 109 minutes; P < .001) and longer median aortic crossclamp times (170 minutes vs 92 minutes; P < .001). Patients who required MCS had more associated VSD repairs (48% vs 39%; P ¼ .27) and more associated aortic arch obstruction repairs (17% vs 7.9%; P ¼ .07). More patients who required MCS weighed <2.5 kg at time of ASO (9.5% vs 3.2%; P ¼ .05). Reintervention for Coronary Stenosis Six patients (0.6%) required 8 reinterventions—all reoperations—on the coronary arteries at a median 11 days (IQR, 6-33 days; range, 1 day to 9 years) after ASO. Three patients with normal coronary anatomy (0.4%; 3 out of 697) required reintervention for coronary stenosis and 3 patients (0.9%; 3 out of 336) with abnormal coronary anatomy. The difference was not statistically significant (P ¼ .40).
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Patient 1 underwent an uneventful ASO for TGA-IVS at 8 days of age with LAD, RCA, and Cx coronary anatomy of 1LAD, 2RCA; Cx, and end-to-end coarctation repair at age 11 days. The patient had a very small, doubly committed VSD that was missed during the ASO. The patient developed chest pain 9 years after ASO and transthoracic echocardiography demonstrated partial occlusion of the LAD by the distorted cusp of the aortic valve. Aortic valve repair was performed, the VSD was closed and LAD was reimplanted. The patient is in NYHA functional class I at age 17 years. Patient 2 underwent an ASO for TGA-IVS at age 8 days with normal coronary anatomy. The patient’s immediate postoperative period was complicated by bradycardia and blood pressure dependent electrocardiogram changes. A coronary angiogram was performed demonstrating patent coronaries; however, there was concern for ongoing coronary ischemia. The patient returned to theatre and underwent relocation of the RCA button. The patient is in NYHA functional class I at age 13 years.
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TABLE 3. Early mortality by decade Era
Anatomy
Early mortality
1983-1989
Overall Normal CA anatomy Abnormal CA anatomy
3.4 (5/148) 4.9 (4/82) 1.5 (1/66)
Overall Normal CA anatomy Abnormal CA anatomy
3.4 (12/352) 3.5 (9/254) 3.1 (3/98)
Overall Normal CA anatomy Abnormal CA anatomy
3.2 (17/533) 2.2 (8/361) 3.1 (9/272)
1990-1999
2000-2013
Values are presented as % (n/N). CA, Coronary artery.
Patient 3 underwent ASO for TGA-IVS at age 18 days with coronary anatomy of 1LAD, RCA; 2Cx. Immediately after coming of bypass, there was ischemia of the posterior ventricular wall. The Cx button was repositioned and the child was supported with extracorporeal membrane oxygenation (ECMO). Unfortunately, the patient had cerebral embolism while on ECMO and died on day 12 after ASO. Patient 4 underwent ASO and end to side arch repair for Taussig-Bing anomaly with coarctation at age 6 days with
normal coronary anatomy. The patient had a cardiac arrest in the intensive care unit and was placed on ECMO. The coronary angiogram demonstrated severe stenosis of the left coronary artery and the left coronary button was relocated lower into the sinus of Valsalva. The patient is alive and well at age 7 years. Patient 5 underwent ASO for TGA-VSD at age 11 days with normal coronary anatomy. The patient went into ventricular tachycardia the day after ASO. Because of concern for right coronary ischemia, the patient returned to operating theatre for reimplantation of the RCA button. The patient is in NYHA functional class I at age 16 years. Patient 6 underwent ASO for TGA-IVS at 11 days of age with coronary anatomy of 1LAD, RCA; 2Cx. The patient was unable to be successfully weaned from cardiopulmonary bypass at time of ASO and was placed on ECMO. A coronary angiogram demonstrated obstruction at the level of the Cx and this was revised with a patch on day 9 postoperatively. Repeat coronary angiography demonstrated residual obstruction at the level of the Cx patch and the coronary was relocated to the nonfacing sinus. The patient was unable to be weaned off ECMO and died at day 23 postoperatively. DISCUSSION An anomalous coronary anatomy may represent a technical challenge during ASO. The most common abnormal anatomy is Cx coronary artery arising from sinus 2.1-3,6,7
Survival (%)
100
75
50
Number at risk Normal CA Abnormal CA Single CA Intramural CA
0
5
10 Years since ASO
15
20
697 250 34 52
544 189 23 43
416 148 19 38
293 102 14 26
106 53 10 14
FIGURE 1. Survival by coronary artery anatomy. Survival for patients with normal coronary arteries (CA) was 96% (95% confidence interval [95% CI], 94%-97%) at 5, 10, and 20 years after arterial switch operation (ASO). Survival for patients with CAs was 96% (95% CI, 92%-98%) at 5, 10, and 20 years after ASO. Survival for patients with single CA was 91% (95% CI, 74%-97%) at 5, 10, and 20 years after ASO. Survival for patients with intramural CAs was 98% (95% CI, 87%-100%) at 5, 10, and 20 years after ASO.
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ASO was performed in 1033 children from 1983–2013
Medical records were retrospectively reviewed
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• Low early mortality • Low rate of coronary reoperation
• Higher early mortality but not statistically significant • Higher rates of postoperative MCS • Low rate of coronary reoperation
FIGURE 2. Normal and abnormal coronary artery outcomes. Abnormal coronary artery anatomy associated with a higher early mortality but was not statistically significant and was still had a low rate of coronary reoperation. ASO, Arterial switch operation; RCA, right coronary artery; LAD, left anterior descending artery; LCx, left circumflex artery; MCS, mechanical circulatory support.
A single coronary artery—or all coronaries arising from the same sinus—are usually the next most common anomaly.1,3, Single coronary artery and intramural coronary arteries are uncommon coronary patterns and, thus, a large cohort of patients is necessary to analyze risk factors for mortality. Technical experience with complex coronary anomalies has improved the results of many institutions over the years. Despite this improvement, contradictory results still exist in the literature. The meta-analysis by Pasquali and colleagues10 on the effect of coronary anatomy on mortality after ASO has been referenced by many.6,7,9,11-16 In this metaanalysis of 1942 patients, any variant of coronary artery anatomy doubled the risk of death. Furthermore, the same meta-analysis demonstrated that risk of death in patients with a single coronary artery was 3 times higher and in those with an intramural coronary artery 7 times higher compared with those with normal coronary anatomy. This metaanalysis was limited to studies of patients undergoing operation between 1977 and 1999. A more recent study by Trezzi and colleagues6 investigated the effect of anomalous coronary anatomy in 283 children undergoing ASO between 2000 and 2015 and found it not to be a risk factor for operative mortality. Outcomes for intramural coronaries have been particularly varied with operative mortality ranging from 0% to 24% in recent studies.9,17,18 Likewise, single coronary arteries have also been a focus. The largest 196
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series of such patients comes from Gerelli and colleagues,11 who published results of 73 children with single coronary arteries undergoing ASO. Mortality was 11%. However, all deaths except 1 occurred in their patients operated before 2001. In our cohort of 1033 patients across 2 institutions, there were 2 (5.9%) operative deaths in patients with a single coronary artery and 1 (1.9%) operative death in patients with intramural coronaries. Both anomalies were not statistically significant risk factors for mortality. The technique for coronary translocation across both our institutions is the trapdoor technique, which has been previously described, and has been consistently used since its introduction by Mee and colleagues during the 1980s.2,18-20 We previously published a detailed description of our technique to translocate intramural coronary arteries.18 We observed higher early mortalities in TGA-IVS and TGA-VSD patients with abnormal coronary anatomy compared with normal coronary anatomy (1.2% vs 2.7% and 3.9% vs 5.8%) in the current study. Despite higher early mortality in children with anomalous coronary patterns, these differences between the groups were not statistically significant nor was anomalous coronary anatomy or any specific anomalous coronary pattern a risk factor on Cox regression analysis (Figure 2). We found that the need for postoperative MCS was more common in patients with abnormal coronary artery anatomy and intramural coronary arteries compared to those with normal coronary anatomy. However, this finding must be tempered by the fact that patients who required MCS had longer cardiopulmonary bypass and aortic crossclamp times, more concomitant VSD and aortic arch obstruction repairs, and were more likely to weigh<2.5 kg. Those factors, leading to more complex repairs and longer ischemic times, may be responsible for the higher need for MCS in these patients. Encouragingly, reinterventions for coronary stenosis were rare in patients (0.6%). Our 2 institutions do not perform routine coronary angiogram in patients after the ASO and do not have set protocols for coronary surveillance after ASO. However, most patients at both institutions undergo exercise stress testing before transfer to adult care. Stress testing that is suggestive of ischemia is followed with myocardial perfusion imaging or coronary angiography. Although no data exists on the ideal form of surveillance, it would be reasonable to routinely do an exercise test in asymptomatic patients with abnormal coronary artery anatomy on transition to adult congenital service. All patients with exercise-induced chest pain or those unable to complete exercise test should undergo computed tomography scan assessment of the coronary arteries. Coronary events are generally reported to be low late after ASO,1,3 but may be more common in institutions where routine follow-up coronary angiography is performed.2,21
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Limitations A limitation of this study was its retrospective nature. Differences in mortality may not be statistically significant due to small number of early deaths. Routine coronary angiography was not performed in our patients and therefore we cannot comment on the potential for coronary stenosis in asymptomatic patients. The rarity of certain types of anomalous coronaries as well as the low early mortality means significant differences in mortality are difficult to detect. CONCLUSIONS Anomalous coronary artery anatomy was not a statistically significant risk factor for mortality after ASO. However, patients with anomalous coronary anatomy had higher early mortality after ASO. Webcast You can watch a Webcast of this AATS meeting presentation by going to: https://aats.blob.core.windows.net/ media/19%20AM/Monday_May6/202BD/202BD/S90% 20-%20Coronary%20artery%20anomalies%20in%20 children/S90_5_webcast_053403526.mp4.
Conflict of Interest Statement Dr Brizard has a financial relationship with Admedus and Dr d’Udekem has financial relationships with Actelion and MSD. All other authors have nothing to disclose with regard to commercial support. References 1. Fricke TA, d’Udekem Y, Richardson M, Thuys C, Dronavalli M, Ramsay JM, et al. Outcomes of the arterial switch operation for transposition of the great arteries: 25 years of experience. Ann Thorac Surg. 2012;94:139-45. 2. Fricke TA, Konstantinov IE. Arterial switch operation: operative approach and outcomes. Ann Thorac Surg. 2019;107:302-10. 3. Khairy P, Clair M, Fernandes SM, Blume ED, Powell AJ, Newburger JW, et al. Cardiovascular outcomes after the arterial switch operation for D-transposition of the great arteries. Circulation. 2013;127:331-9. 4. Wong SH, Finucane K, Kerr AR, O’Donnell C, West T, Gentles TL. Cardiac outcome up to 15 years after the arterial switch operation. Heart Lung Circ. 2008;17:48-53. 5. Fricke TA, Loyer BR, Huang L, Griffiths S, Yaftian N, Dalziel KM, et al. Longterm quality of life in adult survivors after the arterial switch operation. Eur J Cardiothorac Surg. 2018;54:1001-3. 6. Trezzi M, Polito A, Albano A, Albanese SB, Cetrano E, Carotti A. Intraoperative coronary revision but not coronary pattern is associated with mortality after arterial switch operation. Eur J Cardiothorac Surg. 2017;52:83-9. 7. Moll M, Michalak KW, Sobczak-Budlewska K, Moll JA, Kopala M, Szymczyk K, et al. Coronary artery anomalies in patients with transposition of the great arteries and their impact on postoperative outcomes. Ann Thorac Surg. 2017;104:1620-8. 8. Lalezari S, Bruggemans EF, Blom NA, Hazekamp MG. Thirty-year experience with the arterial switch operation. Ann Thorac Surg. 2011;92: 973-9. 9. Metton O, Calvaruso D, Gaudin R, Mussa S, Raisky O, Bonnet D, et al. Intramural coronary arteries and outcome of neonatal arterial switch operation. Eur J Cardiothorac Surg. 2010;37:1246-53. 10. Pasquali SK, Hasselblad V, Li JS, Kong DF, Sanders SP. Coronary artery pattern and outcome of arterial switch operation for transposition of the great arteries: a meta-analysis. Circulation. 2002;106:2575-80. 11. Gerelli S, Pontailler M, Rochas B, Angeli E, Van Steenberghe M, Bonnet D, et al. Single coronary artery and neonatal arterial switch operation: early and longterm outcomes. Eur J Cardiothorac Surg. 2017;52:90-5. 12. Al Anani S, Fughhi I, Taqatqa A, Elzein C, Ilbawi MN, Polimenakos AC. Transposition of great arteries with complex coronary artery variants: time-related events following arterial switch operation. Pediatr Cardiol. 2017; 38:513-24. 13. Machida D, Isomatsu Y, Goda M, Suzuki S, Asou T, Masuda M. Successful coronary transfer for transposition of the great arteries with bilateral intramural coronary arteries from a single aortic sinus. Gen Thorac Cardiovasc Surg. 2018;66: 476-9. 14. Kitamura S. Pediatric coronary artery bypass surgery for congenital heart disease. Ann Thorac Surg. 2018;106:1570-7. 15. Ou P, Khraiche D, Celermajer DS, Agnoletti G, Le Quan Sang K-H, Thalabard JC, et al. Mechanisms of coronary complications after the arterial switch for transposition of the great arteries. J Thorac Cardiovasc Surg. 2013; 145:1263-9. 16. Mavroudis C, Stewart RD, Backer CL, Rudra H, Vargo P, Jacobs ML. Reoperative techniques for complications after arterial switch. Ann Thorac Surg. 2011; 92:1747-54. 17. Thrupp SF, Gentles TL, Kerr AR, Finucane K. Arterial switch operation: early and late outcome for intramural coronary arteries. Ann Thorac Surg. 2012;94: 2084-90. 18. Fricke TA, Bulstra AE, Naimo PS, Bullock A, Robertson T, d’Udekem Y, et al. Excellent long-term outcomes of the arterial switch operation in patients with intramural coronary arteries. Ann Thorac Surg. 2016;101:725-9. 19. Asou T, Karl TR, Pawade A, Mee RBB. Arterial switch-translocation of the intramural coronary artery. Ann Thorac Surg. 1994;57:461-5. 20. Fricke TA, Konstantinov IE. Translocation of intramural coronary artery at the arterial switch operation: divide and conquer? J Thorac Cardiovasc Surg. 2018;155:e131-2.
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Asymptomatic stenosis has been reported in approximately 10% of patients on coronary angiography or computed tomography coronary angiogram15,22 and abnormal coronary artery patterns have been identified as risk factors for coronary obstruction.21,23,24 When late coronary ischemia does occur, surgical coronary revascularization can be performed with a low mortality.16,25-28 The role of percutaneous coronary intervention in growing children is less well defined,14,29 but may play a larger role in adult patients in the future. Our results demonstrate that anomalous coronary arteries are not a risk factor for ASO. Reports of successful ASOs in even the most difficult coronary anatomy have been published, including a single coronary artery from the nonfacing sinus30 and bilateral intramural coronaries from a single sinus.13,31 Despite this, myocardial ischemia is the most common cause of early mortality and, although rare, late death is also coronary-related in many instances.32 Furthermore, the demonstration of early atherosclerosis by intravascular ultrasound in coronaries after ASO33 highlights the need for ongoing coronary surveillance throughout adulthood despite good outcomes.
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21. Legendre A, Losay J, Touchot-Kone A, Serraf A, Belli E, Piot JD, et al. Coronary events after arterial switch operation for transposition of the great arteries. Circulation. 2003;108:186-90. 22. Tsuda T, Bhat AM, Robinson BW, Baffa JM, Radtke W. Coronary artery problems late after arterial switch operation for transposition of the great arteries. Circ J. 2015;79:2372-9. 23. Angeli E, Formigari R, Pace Napoleone C, Oppido G, Ragni L, Picchio FM, et al. Long-term coronary artery outcome after arterial switch operation for transposition of the great arteries. Eur J Cardiothorac Surg. 2010;38: 714-20. 24. Bonhoeffer P, Bonnet D, Piechaud J-F, St€umper O, Aggoun Y, Villain E, et al. Coronary artery obstruction after the arterial switch operation for transposition of the great arteries in newborns. J Am Coll Cardiol. 1997;29: 202-6. 25. Angeli E, Raisky O, Bonnet D, Sidi D, Vouhe PR. Late reoperations after neonatal arterial switch operation for transposition of the great arteries. Eur J Cardiothorac Surg. 2008;34:32-6. 26. Raisky O, Bergoend E, Agnoletti G, Ou P, Bonnet D, Sidi D, et al. Late coronary artery lesions after neonatal arterial switch operation: results of surgical coronary revascularization. Eur J Cardiothorac Surg. 2007; 31:894-8. 27. Jung JC, Kwak JG, Kim ER, Bang JH, Min J, Lim JH, et al. Reoperation for coronary artery stenosis after arterial switch operation. Interact Cardiovasc Thorac Surg. 2018;27:169-76. 28. Mavroudis C, Backer CL, Duffy CE, Pahl E, Wax DF. Pediatric coronary artery bypass for Kawasaki congenital, post arterial switch, and iatrogenic lesions. Ann Thorac Surg. 1999;68:506-12. 29. Kampmann C, Kuroczynski W, Trubel H, Knuf M, Schneider M, Heinemann MK. Late results after PTCA for coronary stenosis after the arterial switch procedure for transposition of the great arteries. Ann Thorac Surg. 2005; 80:1641-6. 30. Konstantinov IE, Fricke TA, d’Udekem Y, Radford DJ. Translocation of a single coronary artery from the nonfacing sinus in the arterial switch operation: longterm patency of the interposition graft. J Thorac Cardiovasc Surg. 2010;140: 1193-4. 31. Cetrano E, Carotti A. Surgical treatment of transposition of the great arteries with bilateral intramural coronary arteries. Ann Thorac Surg. 2012; 93:986-7. 32. Losay J, Touchot A, Serraf A, Litvinova A, Lambert V, Piot JD, et al. Late outcome after arterial switch operation for transposition of the great arteries. Circulation. 2001;104:121-6. 33. Pedra SRFF, Pedra CAC, Abizaid AA, Braga SLN, Staico R, Arrieta R, et al. Intracoronary ultrasound assessment late after the arterial switch operation for transposition of the great arteries. J Am Coll Cardiol. 2005;45: 2061-8.
Key Words: arterial switch operation, anomalous coronary artery
Discussion Dr Jonathan M. Chen (Philadelphia, Pa). The group at Royal Children’s and Queensland Hospitals are truly to be commended on an enviable series of more than 1000 arterial switch operations with outstanding results, and certainly since the era of transesophageal echocardiography, congenital surgeons have come a long way in developing safe and reproducible techniques to overcome the challenges of complex coronary transfer. Single coronary, single ostium,
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double looping, intramural, all these variants had, for the most part, extraordinarily low mortality in this series across 30 years of study. It is really truly remarkable. My only comment would be that in deference to Jan Quaegebeur, there is no such thing as normal anatomy, there’s only usual anatomy. Six patients required surgical coronary reinterventions about 2 weeks after their switch. In retrospect, was there anything about the reinterventions you did that would inform what you should have done the first time around? Dr Tyson Alexander Fricke (Melbourne, Australia). I think the straight answer is no. In those 6 patients, 5 required early reoperation, and in those 5 patients, 3 had normal anatomy and they had what seemed like routine, normal arterial switch but experienced cardiac arrest during the early postop period, usually the first or second day. And on revision, in all 3 of those patients, there was kinking of the left coronary button. But nothing specific that I can identify looking through their original operations and the revision operations that would inform practice. Dr Chen. During the Van Arsdell-Calderone dynasty they suggested the intraoperative revision of coronary anastomoses based on echocardiogram findings in the operating room in terms of flow within the coronaries. Do you routinely do postswitch evaluation with transesophageal echocardiography and/or epicardial, and do you look at the coronary flow? Dr Fricke. To my knowledge, no, but perhaps Dr d’Udekem could elaborate on that. Dr Yves d’Udekem (Melbourne, Australia). We do not check the coronary blood flow at the end of an arterial switch systematically in all patients. Some surgeons in our unit do, some not. If we have the slightest issue, we check these flows with echocardiography and we have a very low threshold to reposition the coronary buttons if any doubt is present. Dr Chen. Fair enough. There are several reports that have been out there that suggest there is a surprisingly high rate of silent coronary occlusion late, like years and years and years after the procedure. Do you have any speculation about what we should think about going forward, what your program’s plans are to interrogate this about the long-term follow-up patients who have undergone arterial switch?
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Dr Fricke. At the Royal Children’s there is no standardized protocol for long-term coronary surveillance. Patients often follow-up in the hospital outpatient clinic, but they are also followed by individual cardiologists, so there is a bit of interclinician variability in their surveillance. Typically, patients will have an exercise stress test in early adolescence, and on transfer from pediatric to adult care, often there is a computed tomography coronary angiogram performed. But neither institution uses routine coronary angiograms. But looking, as you mentioned, in other articles published elsewhere, there are some instances of silent coronary obstruction, so perhaps that is something that needs to be re-evaluated. Dr Chen. Thanks. Great presentation. Dr d’Udekem. If you look at the recent literature, the incidence of silent coronary occlusion is relatively small, 5% at most. But the incidence of symptomatic coronary occlusion is even smaller. It remains difficult to know exactly how to organize the follow-up of these patients. Dr James Jaggers (Aurora, Colo). My question has to do with preoperative imaging. Sometimes we get an echocardiogram and 1 of the coronary arteries isn’t visible, and my reflex response is to get a computed tomography scan because I want to know what it looks like. If the outcomes are equivalent, does it really matter? Can you just go to the operating room and deal with it then? It sounds like if the outcomes are not really different, depending on anatomy, am I being unnecessarily concerned? Dr d’Udekem. Some of our cardiologists ask for a computed tomography scan, but it is variable among cardiologists. I have to say, I was trained in Great Ormond
Street Hospital at the time of Marc de Leval doing his human factors studies. For those who don’t know his study, they were looking at all the different parameters that could influence the outcomes and in particular the psychological factors. He found that if you have a wrong diagnosis of the coronary anatomy, that had an influence on your outcome. Whether the cardiologist identified that it was intramural and actually it was not intramural or if it’s an intramural and it was not expected, in both cases, it influenced the outcome. So, yes, one could wonder whether you should look too closely at the report of the cardiologist if you are not certain that it is adequate. Dr Joseph Forbess (Chicago, Ill). In the patients that you placed on mechanical circulatory support, and clearly that was higher in the abnormal coronaries, were any salvaged with transplant, and if so, were they counted among your survivors? How did you account for them? Dr Fricke. No patients proceeded to transplant. Dr Olivier Ghez. A very quick question. Some patients, of course, will occlude a coronary and be asymptomatic in the long term. Do you do checks if you had difficulties at reimplantation or anything? Do you do regular coronary imaging in cases of difficulty at reimplantation? Even if they do well, they may develop secondarily an obstruction even with being asymptomatic initially. Dr d’Udekem. It has been recommended to follow more closely those who had coronary abnormalities or any issues requiring reimplantation of a coronary artery and that may be the way to go.
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TABLE E1. Survival after arterial switch operation (ASO) by coronary pattern Coronary pattern
5y
10 y
20 y
Normal
96 (94-97)
96 (94-97)
96 (94-97)
Anomalous
96 (92-98)
96 (92-98)
96 (92-98)
Single coronary
91 (74-97)
91 (74-97)
91 (74-97)
Intramural coronary
98 (87-100)
98 (87-100)
98 (87-100)
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Values are presented as % (95% confidence interval).
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