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Ann Thorac Surg 2006;82:973-977
© 2006 The Society of Thoracic Surgeons
Cook Children's Medical Center, University of North Texas, Fort Worth, Texas
Accepted for publication April 27, 2006.
* Address correspondence to Dr Erez, Cook Children's Medical Center, 901 Seventh Ave, Suite 330, Fort Worth, TX 76104 (Email: eerez{at}cookchildrens.org).
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Abstract |
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 Top Abstract IntroductionPatients and MethodsResultsCommentSouthern Thoracic Surgical...References |
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METHODS: Between June 2003 and August 2005, 9 patients (8 males) were diagnosed with anomalous origin of a coronary artery coursing between the great vessels. The mean age was 12 ± 5.8 years (range, 4 months to 23 years). Three patients had an intramural origin of the coronary artery. One infant had a single coronary artery and was diagnosed during follow-up of other heart defects. The 8 older patients all presented with exertional syncope or chest pain and echocardiography was used for the initial diagnosis. Six patients had coronary artery reimplantation in the correct aortic sinus, 2 patients had unroofing of the intramural coronary segment, and 1 patient had pericardial patch enlargement of his right coronary artery before reimplantation. One patient, the infant, awaits repair at an older age.
RESULTS: No postoperative deaths occurred. The mean hospital stay was 5.5 ± 1.2 days (range, 4 to 8 days). Three patients had transient ST segment changes during the first 24 hours postoperatively. Follow-up was 4 months to 2.5 years. All patients underwent an exercise myocardial perfusion scan 3 months postoperatively without evidence of myocardial ischemia, and all patients remain clinically well.
CONCLUSIONS: Echocardiography imaging of young patients with exertional syncope or chest pain is reliable for the diagnosis of this coronary anomaly. It is surgically correctable; however, individual coronary anatomy may cause the surgical approach to vary. The postoperative outcome is excellent.
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Introduction |
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TopAbstractIntroductionPatients and MethodsResultsCommentSouthern Thoracic Surgical...References |
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We have seen a relatively large number of patients within a short period of time at a medium size regional center that were surgically treated for this rare coronary anomaly. Is it a coincidence, or is this anomaly more common than reported? These patients presented with some degree of anatomic variance; however, a proximal segment of a coronary artery was coursing between the great vessels in all patients.
This retrospective clinical study is based on the review of previous reports and our own experience with the diagnosis and surgical treatment of this rare coronary anomaly. We discuss indications for operation, the different surgical approaches as required by the specific anatomic presentation, and possible outcomes.
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Patients and Methods |
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TopAbstractIntroductionPatients and MethodsResultsCommentSouthern Thoracic Surgical...References |
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In 4 patients, the left main coronary artery originated from the right aortic sinus and coursed between the aorta and main pulmonary artery. In the remaining 5 patients, the right coronary artery originated from the left coronary sinus and coursed between the great vessels. Figure 1 demonstrates the different anatomic types of this coronary anomaly as found in the patients that underwent surgical correction. Three patients had an intramural origin of the coronary artery involving slit ostia. One patient had a single anterior coronary artery with the left main origin from the right coronary artery (Fig 2). This patient was the only infant in this group. Diagnosis was made during evaluation for the surgical repair of other heart defects associated with Shone syndrome. The infant underwent repair of severe left ventricular outflow tract obstruction with a posterior malalignment ventricular septal defect. Coronary artery anatomy was confirmed at operation. Repair will occur at an older age.
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In 6 patients, the coronary artery was removed with a button of aortic wall, the proximal part mobilized, and then reimplanted in the correct aortic sinus at the normal anatomic position or slightly higher. One patient had pericardial patch enlargement of the right coronary artery before reimplantation. Meticulous care was taken to avoid tension when repositioning the coronary artery.
The commissure between the right and left coronary cusps in all these patients had to be partially dissected from the aortic wall and subsequently resuspended to the patched aortic wall. A small patch of bovine pericardium similar to the size of the excised aortic coronary artery button was used for the aortic wall reconstruction.
Two patients had unroofing of the intramural coronary segment, which was coursing between the great vessels. Without additional coronary transfer, this unroofing moved the orifice of the coronary artery out from between the great vessels [6]. During the repair, direct coronary perfusion was used to deliver subsequent repeat doses of cardioplegia. After completion of the repair and aortic closure, a final warm dose of blood cardioplegia was again delivered through the aortic root.
The mean cardiopulmonary bypass time was 121 ± 23 minutes (range, 71 to 144 minutes), and the mean aortic cross-clamp time was 93 ± 20 minutes (range, 53 to 111 minutes).
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Results |
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TopAbstractIntroductionPatients and MethodsResultsCommentSouthern Thoracic Surgical...References |
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Mean follow-up was 12 ± 8 months (range, 4 months to 2.5 years). All patients underwent an exercise myocardial perfusion scan 3 months postoperatively. All scans were negative for myocardial ischemia. Those patients who wished to return to athletic activities were allowed to do so. At short-term follow-up, all patients remained asymptomatic.
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Comment |
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TopAbstractIntroductionPatients and MethodsResultsCommentSouthern Thoracic Surgical...References |
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With either left or right anomalous coronary artery coursing between the great vessels, the patient may be asymptomatic and this anomaly only recognized as an incidental finding at autopsy [4]. This has led some authors to recommend surgical intervention only in symptomatic patients [1]. Several other studies, however, reported sudden death in young athletes as the first and only expression of this coronary anomaly [10, 11]. Furthermore, results of the largest autopsy study (32 patients) showed only 37% of the patients were clinically symptomatic before death. Cardiovascular symptoms included syncope, chest pain, and palpitations. The patients who experienced sudden death were young patients compared with the older patient, whose primary cause of death was not cardiac and the coronary anomaly was an incidental finding [2].
Only 1 patient in our series demonstrated abnormal reversible cardiac perfusion scan, with normal ECGs and stress tests. It seems that both exercise stress testing and cardiac perfusion scans have low yield for diagnosis of this anomaly and probably will not change the management of these patients. If positive, they only add strength to the diagnosis.
The previously published data and the fact that most of our group of symptomatic patients did not consistently show ischemic changes on clinical evaluation may suggest a dynamic coronary obstruction phenomenon that may express itself only under certain circumstances involving intense physical exercise. The diagnosis of the coronary artery anomaly with its potential high-risk for sudden death associated with young age and high activity levels led us to decide in favor of a surgical correction. The same approach would probably be adopted in the young asymptomatic patients.
The incidence of this anomaly is 0.05% to 1.2% according to autopsy, angiographic, or prospective echocardiography studies [1, 4, 12]. No studies screening asymptomatic children and adolescents have been performed to accurately assess the incidence of congenital coronary artery anomalies in the general population. Is this anomaly more common than actually diagnosed?
Most of our patients were diagnosed during evaluation of chest pain or a syncopal event by the use of transthoracic echocardiography, and the diagnosis was confirmed by cardiac catheterization. Transthoracic echocardiography proved to be reliable in patients with chest pain when the proximal coronary artery anatomy was specifically studied.
We have diagnosed a relatively large number of patients within a short period of time. This may be related to the increased awareness of the possible coronary anomaly as the cause of the symptoms. We found only one report of a false-negative echocardiographic test that failed to demonstrate the anomaly, and the child eventually experienced sudden death with an autopsy confirmation of the diagnosis [1]. Because of the difficulty with diagnosis, we currently believe all these patients should have a cardiac catheterization before operative intervention.
Most of the patients diagnosed with this anomaly, in our study as well as most of the other reports, were young male patients. Reports in women and elderly patients are less common [4]. It is unclear if it is more common in males [1, 6] or if more males are diagnosed because of their relative majority participating in competitive sports.
Several surgical approaches have been suggested in previous reports, including coronary artery bypass grafting using the saphenous vein [5] or the internal mammary artery [1], reimplantation into the correct sinus of the aorta [9], unroofing of the intramural segment of the coronary artery resulting in modified orifice shape and location [6,7,13,14], and main pulmonary artery translocation [11]. Our surgical approach was directed by the individual anatomic findings, with preference of restoring the normal coronary anatomy when possible. This approach may be because we primarily practice as pediatric cardiac surgeons.
In 6 patients, the left and right coronary artery orifices were separated but located in the same aortic sinus. Our preferred approach in this setting was coronary reimplantation into the correct aortic sinus, followed by bovine pericardial patch closure of the defect in the aortic wall. This approach creates a normal coronary anatomy. In 4 patients, however, the aortic valve commissure between the right and left coronary cusps had to be detached to allow for the coronary excision with subsequently resuspension of the commissure to the reconstructed aorta. This may create a potential risk of damaging the aortic valve function, and long-term follow-up will be necessary before its safety is confirmed.
It is certainly possible for a young adult with this coronary anomaly to be treated by an "adult" cardiac surgeon with a preference to perform a coronary artery bypass grafting as previously described [1, 5]. This may neutralize the concerns about the aortic valve; however, it does raise concerns about competitive flow in the coronary artery system and the palliative nature of this approach.
In 2 patients, the anomalous coronary artery was found to have slit ostia with a proximal intramural segment. We modified the coronary ostium shape and location in these patients by resecting the inner wall of the coronary artery, which effectively changed the location of the coronary artery orifice from between the great vessels.
One patient had a single anterior coronary artery with the left main coronary artery subsequently coursing between the main pulmonary artery and the ascending aorta. This was an infant that was diagnosed during echocardiographic evaluation of his ventricular septal defect and severe left ventricular outflow tract obstruction. One case report presenting the same anatomy suggested translocating the main pulmonary artery toward the left pulmonary hilum to create additional space between the aorta and main pulmonary arteries in a situation were there is a single coronary orifice [11]. It is unclear how much additional space will be created when the left main coronary artery travels along the epicardium of the right ventricle at the base of the main pulmonary artery. Because sudden death is rare in asymptomatic children younger then 10 years old with this coronary anomaly [15], we decided to postpone the surgical treatment to allow for further growth of the infant with anticipation of performing coronary artery bypass grafting to the left coronary system at a an older age if a translocation will not be feasible.
Even though this is a rare coronary anomaly, increased awareness may promote early diagnosis in active young athletes. The large variety of anatomic expression has been addressed by several different surgical techniques, which proved to be safe and reliable, to prevent the risk of sudden death in these patients.
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Southern Thoracic Surgical Association: Fifty-Third Annual Meeting |
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TopAbstractIntroductionPatients and MethodsResultsCommentSouthern Thoracic Surgical...References |
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Manuscripts accepted for the Resident Competition must be submitted to the STSA headquarters office no later than September 15, 2006. The Resident Award will be based on abstract, presentation, and manuscript.
Applications for membership should be completed be September 15, 2006, and forwarded to Chairman of Membership Committee, Southern Thoracic Surgical Association, 633 N Saint Clair St, Suite 2320, Chicago, IL 60611-3658.
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References |
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TopAbstractIntroductionPatients and MethodsResultsCommentSouthern Thoracic Surgical...References |
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This article has been cited by other articles:
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  G. Hamzeh, A. Crespo, R. Estaran, M. A Rodriguez, R. Voces, and J. I Aramendi Anomalous Origin of Right Coronary Artery From Left Coronary Sinus Asian Cardiovasc Thorac Ann, August 1, 2008; 16(4): 305 - 308. [Abstract] [Full Text] [PDF]
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 J. A. Brothers, M. G. McBride, M. A. Seliem, B. S. Marino, R. S. Tomlinson, M. H. Pampaloni, J. W. Gaynor, T. L. Spray, and S. M. Paridon Evaluation of Myocardial Ischemia After Surgical Repair of Anomalous Aortic Origin of a Coronary Artery in a Series of Pediatric Patients J. Am. Coll. Cardiol., November 20, 2007; 50(21): 2078 - 2082. [Abstract] [Full Text] [PDF]
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