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Ann Thorac Surg 1999;68:506-512
© 1999 The Society of Thoracic Surgeons

Original Articles

Pediatric coronary artery bypass for Kawasaki, congenital, post arterial switch, and iatrogenic lesions

Constantine Mavroudis, MDa, Carl L. Backer, MDa, C. Elise Duffy, MDa, Elfriede Pahl, MDa, David F. Wax, MDa

a Department of Cardiovascular Surgery, Children’s Memorial Hospital, Chicago, Illinois, USA

Address reprint requests to Dr Mavroudis, Department of Cardiovascular Surgery, Children’s Memorial Hospital, 2300 Children’s Plaza, MC22, Chicago, IL 60614
e-mail: c-mavroudis{at}nwu.edu

Presented at the Thirty-fifth Annual Meeting of The Society of Thoracic Surgeons, San Antonio, TX, Jan 25–27, 1999.


   Abstract
Top
 Abstract
Introduction
 Material and methods
 Results
 Comment
 References
 
Background. Pediatric coronary artery bypass (PCAB) has been recently employed for expanding indications to treat acquired, congenital, post arterial switch, and other iatrogenic pediatric coronary artery problems.

Methods. Between 1987 and 1998, 3 infants and 13 children (n = 16, mean age 6.1 years, range 2 months–18 years) underwent one or more internal thoracic artery (ITA) to coronary artery (CA) bypass grafts for Kawasaki disease (n = 4), congenital lesions (n = 3), post arterial switch (n = 4), and other iatrogenic obstructions (n = 5). Proximal left main CA arterioplasty was performed concurrently with ITA-CA bypass in 4 patients.

Results. Survival is 93.8%. All bypass grafts in surviving patients are patent 2 months–11 years postoperation. The 11 elective patients are well (NYHA I–II). The 5 emergent operations were performed in 2 infants and 3 adolescents who had poor ventricular function prior to ITA-CA bypass due to iatrogenic injuries in 3, congenital critical left main stenosis in 1, and intraoperative iatrogenic coronary injury in 1. The 3 adolescents fared worse, resulting in death in the first, cardiac transplantation in the second, and full recovery in the third. The 2 infants have steadily improving ventricular function.

Conclusions. ITA-CA bypass can be successfully performed in infants and children for expanding elective and life-saving indications with excellent results. Poor preoperative ventricular function often persists, especially in those older children with iatrogenic injuries, and may result in death or cardiac transplantation.


   Introduction
Top
 Abstract
 Introduction
Material and methods
 Results
 Comment
 References
 
Pediatric coronary artery (CA) bypass has been used with increasing frequency for patients with Kawasaki disease [1, 2], congenital lesions [3–12], post arterial switch [13–17], and other iatrogenic obstructions [18, 19]. In addition, operations which require manipulation and reimplantation of coronary arteries, such as with arterial switch or the Ross procedure, establish a patient base which is at risk for acute or chronic ischemic complications [8, 9, 20]. Coronary artery bypass has been shown to favorably impact the clinical course of those patients with coronary insufficiency [2, 9]. In particular, the internal thoracic artery (ITA) to coronary artery (CA) bypass graft has a high patency rate as well as demonstrated anastomotic and linear growth potential [1, 2].

We previously published our initial results with CA bypass in infants and children with emphasis on expanding indications and favorable size dimensions in small children [9]. The purpose of this paper is to review our continued experience with ITA to CA bypass for expanded indications, to document the mid-term patency rate, and to evaluate the functional results.


   Material and methods
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 Abstract
 Introduction
 Material and methods
Results
 Comment
 References
 
Between 1987 and 1998, 16 infants and children with congenital and acquired coronary lesions underwent ITA to CA bypass for Kawasaki disease (n = 4) (Table 1 ), congenital lesions (n = 3) (Table 2 ), post arterial switch (n = 4) (Table 3 ), and other iatrogenic obstructions (n = 5) (Table 4).


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  		Table 1. Coronary Bypass for Kawasaki Disease (n = 4)

 

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  		Table 2. Coronary Bypass for Congenital Lesions (n = 3)

 

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  		Table 3. Coronary Bypass Postarterial Switch (n = 4)

 

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  		Table 4. Coronary Bypass for Other Iatrogenic Lesions (n = 5)

 
There were 8 males and 8 females; the mean age was 8.7 ± 5.8 years with a range of 2 months to 18 years. Diagnosis was established by various combinations of coronary angiography, thallium perfusion scan, dobutamine stress echocardiography, and findings at operation. All patients had a right and/or left ITA to affected CA bypass. The average CA size (measured at operation) was 1.65 ± 0.55 mm (range 0.7–2 mm) and the average ITA size was 1.43 ± 0.32 mm (range 0.8 mm–2 mm). One patient had an additional reversed saphenous vein (RSV) bypass graft. Proximal left main CA arterioplasties were performed in 4 patients in addition to distal CA bypass; concomitant corrective operations such as valve replacement, ventricular septal defect closure, pulmonary artery arterioplasty, and the like were performed in 8 patients. Redo sternotomy was required in 8 patients and had no negative effect on ITA dissection. The conduct of the operation was similar in all patients except the child with anomalous left coronary artery from the pulmonary artery, who was operated on through a left thoracotomy without cardiopulmonary bypass early in the series before aortic reimplantation had been proven successful. The rest of the patients had median sternotomy, hypothermic cardiopulmonary bypass (28°C), antegrade and retrograde administration of cold blood cardioplegic solution, and a left ventricular vent. Standard techniques were used for ITA dissection (topical papaverine); anastomoses were accomplished with either 7-0 or 8-0 monofilament running or interrupted suture techniques, depending on the size of the arteries.

Follow-up comprised of early postoperative coronary angiography in 15 of 16 patients. Repeat postoperative coronary angiography was performed in six patients. Five patients had postoperative dobutamine stress echocardiography; 3 patients had postoperative nuclear perfusion scans; and 5 patients had postoperative exercise stress tests.


   Results
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 Abstract
 Introduction
 Material and methods
 Results
Comment
 References
 
The pertinent clinical features of our patients are noted in Tables 1–4. There was one death (6.3%) in a 10-year-old boy who had an aortic annular tear and left main CA dissection due to transcatheter balloon aortic valvuloplasty. He had emergency double CA bypass (left ITA to left anterior descending (LAD), RSV to obtuse marginal (OM) coronary arteries), aortic valve replacement (#17 St. Jude HP valve; St. Jude Medical Inc, St. Paul, MN), and left ventricular assist (left atrium to aorta, Biomedicus pump). He was weaned from left ventricular assistance but died while on the transplant list on postoperative day 55. Major complications occurred in 4 surviving patients: (1) a 3-month-old with critical left main stenosis and mitral regurgitation required an open sternum (silicone elastomer skin patch) with prolonged inotropic and ventilatory support; (2) a 2-month-old s/p arterial switch, extracorporeal membrane oxygenation (ECMO), and left main CA occlusion at another institution required an open sternum (silicone elastomer skin patch) with prolonged inotropic and ventilatory support after revascularization and ventricular septal defect (VSD) closure; (3) a 14-year-old s/p multiple operations for truncus arteriosus and iatrogenic left main CA injury at another institution responded poorly to revascularization and conduit change which required subsequent orthotopic cardiac transplantation; and (4) an 18-year-old with Marfan disease required prolonged cardiopulmonary bypass due to unwanted intraoperative right RCA avulsion and subsequent CA bypass. The average length of stay was 16.6 ± 19.1 days (range 3–56 days). When the 4 patients with extended length of stay (56, 25, 50, and 55 days) are censored, the average length of stay was 6.7 ± 2.6 days. Fifteen patients (including the one who died), underwent timely postoperative coronary angiography, which showed anastomotic patency in all grafts without evidence of significant stenosis, kinking, or delayed filling. Repeat (second time postoperative angiography) was performed in 6 patients 27.6 ± 12.7 months postoperatively. "String signs" [21] were identified in 3 patients; two in patients who had both proximal left main arterioplasty and distal CA bypass; and in 1 patient with Kawasaki disease who had natural recanalization and remodeling of the proximal RCA well after CA bypass. Postoperative electrocardiograms in surviving patients showed no evidence (new Q waves) of a perioperative myocardial infarction.


   Comment
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 Abstract
 Introduction
 Material and methods
 Results
 Comment
References
 
Our continued application of CA bypass in infants and children for expanded indications has resulted in favorable mid-term outcomes that relate to graft patency and functional class. These findings mirror previous reports in patients with Kawasaki disease [1, 2] and in patients with other forms of acquired and congenital coronary insufficiency [3, 6, 13–15, 18].

The most common indication for CA bypass in children is coronary ischemia due to Kawasaki disease [1, 2]. Our data are similar to others who have shown successful revascularization and high patency rates [1, 2]. Two of our patients are now 18 and 15 years old, 5 and 10 years after CA bypass respectively, with patent grafts and excellent stress perfusion studies without ischemia. Another patient developed a critical stenosis of the RCA within months of his acute illness; he underwent successful CA bypass at 8 months of age and is thought to be one of the youngest patients to have undergone revascularization for Kawasaki disease. A follow-up study at age 3 years demonstrated significant angiographic improvement with almost complete resolution of the giant aneurysm and recanalization of the thrombus, as well as remodeling of the RCA with only mild residual stenosis. His right ITA to RCA graft, which was widely patent at the immediate postoperative catheterization study, is now patent but diffusely small with development of a "string sign" [21] indicating that the predominant distal flow is from the native remodeled RCA. It could be argued that RITA to RCA bypass was unnecessary in this setting. However, Kato and colleagues [22] have noted that the early period after acute illness and giant aneurysm formation poses the highest risk for myocardial infarction and death. Furthermore, the majority of these cardiac events occurred at rest. Long-term follow-up studies by this group [23] showed that myocardial infarction occurred in more than one-third of patients who developed coronary ischemia. For these reasons, we believe that it is appropriate to subject these patients with appropriate findings to CA bypass in light of the excellent postoperative results, even if long-term self-reparative and remodeling events can be anticipated in a certain number of patients. Our present indications for CA bypass include: (1) symptomatic patients with severe CA disease consisting of giant aneurysms and significant CA artery stenosis (> 50% stenosis) and (2) asymptomatic patients who have inducible ischemia during non-invasive testing and/or angiographic progression of significant CA stenoses.

Congenital CA lesions are being diagnosed with increasing frequency and certainty due to a greater awareness of clinicopathologic entities that can result in myocardial infarction and sudden death [11]. Anomalies such as CA fistula [10], anomalous origin of the left main CA from the pulmonary artery [12], and intramural CA [24] are best treated by ligation, aortic reimplantation, and supra-arterial myotomy, respectively. Only rarely is CA bypass required. Various types of single CA associated with an abnormal course between the great vessels have been identified as high risk for untoward coronary events [7, 8, 11]. Depending on the anatomy, proximal CA arterioplasty [25] or CA bypass [9], as was performed in our patient, is indicated. Left main CA atresia/critical stenosis has been treated by proximal arterioplasty, CA bypass, or both [3–5, 9]. From the few cases that have been reported, it is difficult to draw conclusions. However, it appears that our patient was better served by proximal coronary arterioplasty since the ITA-LAD graft developed a "string sign" due to competitive forward flow through the native vessel.

Coronary ischemic complications at the time of neonatal arterial switch for transposition of the great vessels have been successfully treated by proximal pericardial patch coronary arterioplasty [26] and CA bypass [13–15]. Early predictions of possible long-term coronary ischemic complications have been realized [17], resulting in successful redo operations to perform proximal coronary arterioplasty [27]. We have experience with both short- and long-term ischemic complications after the arterial switch operation that raises some interesting questions about the method of coronary revascularization. Our infant case was referred for cardiac transplantation after a failed arterial switch operation and ECMO due to iatrogenic left main CA occlusion. Instead, we performed VSD closure and LITA-LAD bypass. The patient improved, most likely due to VSD closure, because left ventricular function improved only marginally. The LITA-LAD graft remains patent with excellent run-off 8 months postoperatively. The patient’s fate depends on the regenerative potential of the immature myocardium, which if not substantial, will result in cardiac transplantation some day. The other 3 patients, with coronary ischemia long after the arterial switch operations, present revascularization choices that include: proximal arterioplasty, CA bypass, or both. We decided on concomitant arterioplasty-CA bypass for a number of reasons: (1) while proximal arterioplasty seemed the better choice, neointimal hyperplasia has been reported [28–30] in adult patients with atherosclerosis and could complicate the repair by restenosis; (2) stenoses extending into the branch coronary arteries are difficult to repair and are at greater risk for thrombosis [30]; and (3) the ITA-CA bypass graft has an established dependability even in the setting of noncritical proximal stenoses [21]. All 3 of our patients had early postoperative angiography showing: excellent arterioplasty after levorotation in one; excellent patch arterioplasty which extended into the left main CA in the second; and excellent proximal patch arterioplasty with mild left main CA stenosis distal to the patch repair in the third. All three ITA-LAD grafts were widely patent with excellent run-off. Follow-up arteriography (second time postoperative catheterization) was performed in the levorotation arterioplasty patient (no augmentation patch), which showed an emerging ITA-LAD "string sign" with continued wide patency of the proximal arterioplasty repair. The remaining 2 patch augmentation patients have not had additional follow-up studies. It is difficult to draw conclusions based on these limited follow-up data. Proximal arterioplasty has been advocated without concomitant CA bypass with excellent short-term results [27]. Long-term angiographic assessment of restenosis has not been reported. Some authors [28–30] have reported restenosis after left main arterioplasty in adults using various patch materials which eventually required CA bypass. Other authors [31] have demonstrated excellent results with proximal left main patent arterioplasty. It could be that the patch material might play an important role in this setting. We chose to use native aortic or pulmonary artery wall for the patch augmentation. The type of material that is best applicable for this purpose however, is best left to the physiology laboratory to solve. A concomitant ITA-CA bypass after late ischemic complications of the arterial switch operation is clearly controversial. From our limited experience, proximal arterioplasty seems to be adequate as long as the stenosis does not extend into the distal left main CA or its branches. Under these or other questionable circumstances, concomitant ITA-CA bypass seems appropriate therapy until the long-term outcomes of proximal patch augmentation are assessed.

CA bypass for iatrogenic ischemic complications has been extensively studied in the setting of failed percutaneous CA balloon angioplasty [32]. In the greater number of cases, the shorter the time interval to revascularization, the better the outcome. Certainly, this tenet should be no different in infants and children despite the smaller but definitely "bypassable" CAs [9]. The other issue involves distant iatrogenic CA occlusive events with residual problems of ventricular dysfunction, ventricular arrhythmias, and CA fistulas. In general, we prefer revascularization and reparative strategies for this group to rescue "hibernating myocardium" [33] and optimize the chances for recovery, recognizing the possibility of eventual cardiac transplantation.

We have shown that pediatric ITA-CA bypass is an effective and often life-saving therapy for expanding indications in infants and children. The graft patency rate is high and extends into the mid-term follow-up period. Poor preoperative ventricular function often persists, especially in those older children with iatrogenic injuries, and may result in death or cardiac transplantation. Concomitant proximal left main CA arterioplasty and ITA-CA is controversial and will require long term follow-up to determine efficacy. Preoperative identification of hibernating myocardium will help to establish coronary revascularization options in patients with chronic myocardial ischemia and the complications relating thereto.


   References
Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 References
 

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