Congenital heart disease involving the coronary artery

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1 Anomalous Coronary Artery With Aortic Origin and Course Between the Great Arteries: Improved Diagnosis, Anatomic Findings, and Surgical Treatment Eldad Erez, MD, Vincent K. H. Tam, MD, Nancy A. Doublin, MSN, and Jeanie Stakes, RN Cook Children s Medical Center, University of North Texas, Fort Worth, Texas Background. Anomalous origin of a coronary artery with subsequent coursing between the great vessels is a rare congenital heart defect that may cause myocardial ischemia and sudden death. Because of the fatality rate of this anomaly, many are diagnosed at the postmortem examination, and reports of surgical correction are few. We present our experience with the diagnosis and surgical treatment of this rare coronary anomaly. 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 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 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. (Ann Thorac Surg 2006;82:973 7) 2006 by The Society of Thoracic Surgeons Congenital heart disease involving the coronary artery problem as the principal lesion, specifically, anomalous origin of a coronary artery with subsequent coursing between the ascending aorta and the main pulmonary artery, is rare but clinically significant. Myocardial ischemia, syncope, and sudden death associated with exercise in patients with this anatomy have been reported [1, 2]. Because of the high fatality rate associated with this anomaly, most published reports involving larger groups of patients are postmortem diagnoses [3 5]. There are few reports of surgical correction [6, 7], and most of them are case reports [8, 9]. 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 Accepted for publication April 27, Address correspondence to Dr Erez, Cook Children s Medical Center, 901 Seventh Ave, Suite 330, Fort Worth, TX 76104; eerez@ cookchildrens.org. 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. Patients and Methods Between June 2003 and August 2005, 9 patients (8 males) were diagnosed with anomalous aortic origin of a coronary artery coursing between the ascending aorta and main pulmonary artery and were retrospectively studied. The Cook Children s Medical Center Institutional Review Board approved this study on March 28, 2006, and individual consent for the study was waived. The mean age was years (range, 4 months to 23 years). In 4 patients, the left main coronary artery originated 2006 by The Society of Thoracic Surgeons /06/$32.00 Published by Elsevier Inc doi: /j.athoracsur

2 974 EREZ ET AL Ann Thorac Surg ANOMALOUS CORONARY ARTERY 2006;82:973 7 Fig 1. The different anatomic types of this coronary artery anomaly found during surgical repair. AO aorta; CX circumflex artery; LAD left anterior descending artery; LMCA left main coronary artery; RCA right coronary artery; PA pulmonary artery. 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 Fig 2. The anatomy of this coronary artery anomaly in the neonate with a single coronary artery. AO aorta; CX circumflex artery; LAD left anterior descending artery; LMCA left main coronary artery; RCA right coronary artery; PA pulmonary artery. 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. The 8 older patients all had clinical symptoms of exertional syncope or chest pain. Only one patient demonstrated ischemia on a cardiac perfusion scan. All had normal results from electrocardiograms (ECGs) and exercise tolerance tests that were performed as part of their clinical evaluation. The initial diagnosis was made using transthoracic echocardiography, specifically looking at the coronary anatomy. Cardiac catheterization (Fig 3) confirmed the diagnosis. A brief overview of our surgical technique is warranted. The surgical technique for the coronary artery repair consisted of standard cardiopulmonary bypass using two caval cannulae and a single aortic arterial inflow cannula. After initiation of cardiopulmonary bypass, the aorta was clamped and the first dose of cold blood cardioplegia was delivered through the aortic root. The aorta was then opened above the sinotubular junction and the coronary anatomy was defined. 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

3 Ann Thorac Surg EREZ ET AL 2006;82:973 7 ANOMALOUS CORONARY ARTERY 975 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. Fig 3. An angiography frame demonstrates a patient with the right coronary artery originating from the left aortic sinus and coursing between the great vessels. 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 minutes (range, 71 to 144 minutes), and the mean aortic cross-clamp time was minutes (range, 53 to 111 minutes). Results No patients died postoperatively. The mean hospital stay was days (range, 4 to 8 days). Intensive care unit stay was less then 48 hours, and no inotropic support was needed. Three patients had transient ST segment changes during the first 24 hours postoperatively without significant change in their serum troponin levels (all within normal range). Echocardiography demonstrated good flow in the coronary artery system with normal cardiac function. The patients had a normal ECG before discharge. They were discharged on aspirin treatment for 3 months. Comment The clinical significance of a relatively rare coronary anomaly where the left coronary artery arises from the right aortic sinus and courses between the aorta and pulmonary artery was first reported in A study of 13 autopsy findings demonstrated a high frequency of sudden death related to this not-so-minor coronary anomaly [3]. Over the years, the anomalous origin of the right coronary artery from the left aortic sinus with subsequent coursing between the great vessels has also been recognized as a potential risk for chest pain, syncope, and sudden death [4, 5]. 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

4 976 EREZ ET AL Ann Thorac Surg ANOMALOUS CORONARY ARTERY 2006;82:973 7 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. References 1. Davis JA, Cecchin F, Jone TK, Portman MA. Major coronary artery anomalies in a pediatric population: Incidence and clinical importance. Ann Thorac Surg 1994;58: Basso C, Maron BJ, Corrado D, Thiene G. Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes. J Am Coll Cardiol 2000;35: Cheitlin MD, DeCastro CM, McAllister HA. Sudden death as a complication of anomalous left coronary origin from the anterior sinus of Valsalva. A not-so-minor congenital anomaly. Circulation 1974;50: Kragel AH, Roberts WC. Anomalous origin of either the right or left main coronary artery from the aorta with subsequent coursing between aorta and pulmonary trunk: analysis of 32 necropsy cases. Am J Cardiol 1988;62: Roberts WC, Siegel RJ, Zipes DP. Origin of the right coronary artery from the left sinus of Valsalva and its functional consequences: analysis of 10 necropsy patients. Am J Cardiol 1982;49: Frommelt PC, Fommelt MA, Tweddell JS, Jaquiss RDB. Prospective echocardiographic diagnosis and surgical repair of anomalous origin of a coronary artery from the opposite sinus with an interarterial course. J Am Coll Cardiol 2003; 42: Romp RL, Herlong JR, Landolfo CK, et al. Outcome of unroofing procedure for repair of anomalous aortic origin of left or right coronary artery. Ann Thorac Surg 2003;76:589 95; discussion Thomas D, Salloum G, Montalescot G, Drobinski J, Artigou Y, Grosgogeat Y. Anomalous coronary arteries coursing

5 Ann Thorac Surg EREZ ET AL 2006;82:973 7 ANOMALOUS CORONARY ARTERY between aorta and pulmonary trunk: clinical indications for coronary artery bypass. Eur Heart J 1991;12: Rogers SO, Leacche M, Mihaljevic T, Rawn JD, Byrne JG. Surgery for anomalous origin of the right coronary artery from the left aortic sinus. Ann Thorac Surg 2004;78: Maron BJ, Roberts WC, McAllister MA. Sudden death in young athletes. Circulation 1980;62: Rodefeld MD, Culbertson CB, Rosenfeld HM, Hanley FL, Thompson LD. Pulmonary artery translocation: a surgical option for complex anomalous coronary artery anatomy. Ann Thorac Surg 2001;72: Engel HJ, Torres C, Page HL. Major variations in anatomical origin of the coronary arteries: angiographic observations in patients without associated congenital heart disease. Cathet Cardiovasc Diagn 1975;1: Mustafa I, Radley-Smith R,Durrer S, Yacoub MH. Anomalous origin of the left coronary artery from the anterior aortic sinus: a potential cause of sudden death. J Thorac Cardiovasc Surg 1981;82: Nelson-Piercy C, Rickards AF, Yacoub MH. Aberrant origin of the right coronary artery as a potential cause of sudden death: successful anatomical correction. Br Heart J 1990;64: Barth CW, Bray M, Roberts WC. Sudden death in infancy associated with origin of both left main and right coronary arteries from a common ostium above the left sinus of Valsalva. Am J Cardiol 1986;57: Southern Thoracic Surgical Association: Fifty-Third Annual Meeting The Fifty-Third Annual Meeting of the Southern Thoracic Surgical Association (STSA) will be held November 9 11, 2006, in Tucson, Arizona. Manuscripts accepted for the Resident Competition must be submitted to the STSA headquarters office no later than September 15, 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 Please visit the STSA ( or CTSNet ( websites for detailed information on submitting abstracts. All abstracts must be submitted electronically for consideration by The Society of Thoracic Surgeons Ann Thorac Surg 2006;82: /06/$32.00 Published by Elsevier Inc