Outcome of Unroofing Procedure For Repair of Anomalous Aortic Origin of Left or Right Coronary Artery

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1 Outcome of Unroofing Procedure For Repair of Anomalous Aortic Origin of Left or Right Coronary Artery Robert L. Romp, MD, J. René Herlong, MD, Carolyn K. Landolfo, MD, Stephen P. Sanders, MD, Coleen E. Miller, PNP, Ross M. Ungerleider, MD, and James Jaggers, MD Department of Pediatric Cardiovascular Surgery, Duke University Medical Center, Durham, North Carolina, Department of Pediatric Cardiology, The University of Alabama, Birmingham, Alabama, and Department of Pediatric Cardiac Surgery, Oregon Health Sciences University, Portland, Oregon Background. Anomalous aortic origin of a coronary artery (AAOCA) from an incorrect sinus of Valsalva is a relatively rare congenital defect and is associated with sudden death. Several surgical techniques have been described to address this defect, but functional outcome has never been addressed. In this report we evaluate a consecutive series of patients treated with unroofing techniques using transthoracic echocardiography, graded exercise testing, and stress echocardiography to assess functional repair. Methods. Nine consecutive patients (range 7 to 65 years) underwent surgical repair of AAOCA from 1995 to In all patients the intramural segment was at or below the level of the commissure. All patients underwent a modified unroofing to move the coronary artery orifice to the appropriate sinus. In 2 patients, a new orifice was created without significant unroofing and disruption of the commissure. Patients were evaluated prospectively with exercise electrocardiography testing and by resting and stress echocardiography. Results. Of the 9 patients, 8 presented with symptoms suggestive of ischemia (chest pain, dyspnea on exertion, or syncope). Six patients had anomalous left main coronary artery arising from the right sinus of Valsalva, and 3 patients had anomalous right coronary artery from the left sinus of Valsalva. Transthoracic echocardiography and graded exercise testing was performed in all 9 patients (mean 29 months, range 4 to 85 months) after repair. Of the 9 patients, 8 also underwent stress echocardiography. In 8 of 9 patients the newly created coronary artery ostium was visualized by either twodimensional echocardiography or color flow Doppler. All patients were symptom free at the time of follow-up. Exercise stress echocardiography was negative in all patients. Of the 8 patients, 7 had normal left ventricular. No patients had regional wall motion abnormalities suggestive of ischemia. All patients were intervention free except 1 patient who developed severe aortic insufficiency and underwent a subsequent Ross 44 months after his initial. Conclusions. Anomalous origin of a coronary artery from an incorrect sinus of Valsalva is known to be associated with increased risk of sudden death. Surgical correction can be carried out with minimal risk and good anatomic and functional results. Manipulation of the commissure can be avoided by creation of a neo-ostia without extensive unroofing of the intramural segment or manipulation of the intercoronary commissure. This may avoid aortic valve malfunction. (Ann Thorac Surg 2003;76:589 96) 2003 by The Society of Thoracic Surgeons Presented at the Forty-ninth Annual Meeting of the Southern Thoracic Surgical Association, Miami Beach, FL, Nov 7 9, Address reprint requests to Dr Jaggers, Pediatric Cardiovascular Surgery, Duke University Medical Center, Box 3474, Durham, NC 27710; jagge003@mc.duke.edu. Anomalous aortic origin of a coronary artery (AAOCA) from the incorrect coronary sinus of Valsalva is a rare congenital cardiac defect that is associated with increased risk of sudden death [1 3]. The left main coronary artery can arise anomalously from the right sinus (anomalous origin of the left main coronary artery [ALMCA]), or the right coronary artery from the left sinus (anomalous origin of the right coronary artery [ARCA]). In most of these defects, the anomalous coronary artery takes an intramural course between the great arteries and leaves the aortic wall from the appropriate coronary sinus of Valsalva. Many times this anomalous artery shares a common orifice with the other coronary artery. The combined incidence of these defects approximates 0.17% in autopsy series and 0.1% to 0.3% in patients undergoing catheterization or echocardiography [1 10]. Sudden death can occur with either defect, most notably in (but not limited to) young, asymptomatic athletes [1, 2]. In a post mortem study, the incidence of sudden death was 54% among 49 cases of ALMCA and 25% among cases of ARCA [1]. Reports of surgical therapy have been primarily anecdotal with little information regarding the efficacy of surgery and alleviation of potential ischemia. Therefore, this study was undertaken to 2003 by The Society of Thoracic Surgeons /03/$30.00 Published by Elsevier Inc PII S (03)

2 590 ROMP ET AL Ann Thorac Surg OUTCOME OF UNROOFING PROCEDURE 2003;76: evaluate the efficacy of the surgical therapy for AAOCA in the current era of cardiac surgery. Material and Methods A review the Duke University Congenital Cardiovascular Surgery database identified patients with surgically repaired coronary artery anomalies. These patients were then prospectively evaluated after approval by the Duke University Medical Center Institutional Review Board. Eligible patients included those with an anomalous aortic origin of a coronary artery (AAOCA) in which the coronary artery ostium was located in the incorrect sinus of Valsalva and the proximal coronary artery had an intramural course between the aortic root and the pulmonary trunk. Patients with anomalous origin of a coronary artery from the pulmonary artery, fistulous connections of a coronary artery, and coexisting congenital heart defects were excluded. The medical records and preoperative diagnostic testing of all patients were reviewed. A series of 9 consecutive patients was identified and all patients agreed to participate in this study. Only 2 of these patients had had preoperative stress testing for the evaluation of ischemia. In both instances the preoperative stress tests were negative. Enrolled patients underwent prospective anatomic and functional testing including transthoracic echocardiography, graded exercise testing, and stress echocardiography. The transthoracic echocardiograms were performed in the Pediatric Echocardiography Laboratory to visualize the surgically created coronary artery ostium and to determine patency as evidenced by two-dimensional imaging and color flow mapping. The left ventricular dimensions and function were assessed and the aortic valve was evaluated for evidence of insufficiency. Graded exercise testing was performed in the Adult Cardiology Exercise Laboratory using one of two modalities. Treadmill testing was performed following a Bruce protocol. Upright stationary bicycling was performed using a protocol in which freewheel measured wattage was increased every 2-min (intervals of 25, 35, 50, 70, and 90 W). The duration of exertion, blood pressure, and heart rate changes, and presence of Q-wave or ST segment changes suggestive of ischemia were recorded. Adequacy of exercise testing was based upon achievement of a target heart rate calculated to be 85% of the age-based maximal heart rate. The treadmill and stationary bicycle also served as the modality of nonpharmacologic stress for the stress echocardiography testing, which was performed in the Adult Cardiology Echocardiography Laboratory. Images of the left ventricle in the parasternal and apical views were obtained before exertion and immediately after achievement of the threshold heart rate. Patients were evaluated using the American Society of Echocardiography 16-segment model for evidence of wall motion abnormalities suggestive of ischemia (Table 1). Statistical Analysis Descriptive statistics were used for this study and expressed as mean the standard error of the mean. Results The study population consisted of 9 patients who underwent a surgical repair of AAOCA from April 1995 to June 2001 (Table 1). Of the patients, 6 were diagnosed with ALMCA and 3 with ARCA. There were 6 male and 3 female patients. At the time of surgery, the patients ranged in age from 7.2 to 65.2 years (mean 21.0 years, median 11.4 years). Eight of the patients presented with symptoms suggestive of ischemia. One patient was asymptomatic at the time of diagnosis. This patient has the diagnosis of pseudoxanthoma elasticum, which is associated with significant cardiovascular manifestations including premature arteriosclerosis. In the case of this patient, the diagnosis of ARCA was made by screening echocardiography. Surgical repair was recommended because of the potential for development of arteriosclerosis at the site of the anomalous origin of the coronary artery. Follow-up postoperative testing was performed in all 9 patients by means of transthoracic echocardiography and graded exercise testing. Eight of the 9 patients also underwent stress echocardiography. The time from surgery to follow-up testing ranged from 4 to 85 months, with a mean of 29.3 months. Surgical Repair Surgical repair was carried out with standard cardiac surgical techniques. All patients were approached with the intent to repair the coronary artery with an unroofing, as initially described by Mustafa and colleagues [11]. After induction of general anesthesia and full sternotomy, mild hypothermic cardiopulmonary bypass (32 to 34 C) was initiated with distal aortic and right atrial cannulation. Antegrade cardioplegia was used in all cases. After the heart was arrested and cold, aortotomy was performed. In 7 of 9 patients the intramural segment was below the level of the commissure, and repair was accomplished in the first 5 of those 7 patients by unroofing the intramural segment with detachment and resuspension of the intercoronary commissure (Fig 1). In the last 2 patients with the intramural segment below the level of the commissure, a neo-ostium was created without disruption of the commissure or significant unroofing of the intramural segment (Fig 2). In this technique a probe or clamp is passed through the intramural segment into the correct sinus. A neo-ostium is created in the correct sinus, at point at which the coronary artery leaves the aortic wall. The intimal edges are tacked down with fine monofilament suture. In both of these patients the anomalous left coronary artery arose from a common orifice with the RCA. The slit-like orifice of the anomalous artery was not addressed. In 2 patients in whom the intramural segment was above the level of the commissure, the intramural segment in the left sinus was simply unroofed and a new orifice created in the appropriate sinus without manipulation of the commissure. One patient (aged 39 years) with both ALMCA and a severe stenosis of the proximal right coronary artery underwent both an unroofing of the anomalous left coronary and bypass grafting of the right coronary artery

3 Table 1. Patient Data and Function Study Results Patient No. Dx Symptoms Preoperative Tests 1 ALMCA syncope Echo-ALMCA (2/24/95) Tilt-PVC s (2/27/95) Cath-ALMCA (3/6/95) 2 ALMCA CAD CP Cath-ALMCA/RCA stenosis (8/8/96) 3 ALMCA syncope Echo-ALMCA (12/5/97) Cath-ALMCA (12/9/97) 4 ALMCA CP Cath/stress test (8/98) Cath-ALMCA (DUMC 10/8/98) 5 ALMCA CP/syncope Echo-ALMCA (6/21/00) Cath-ALMCA (8/23/00) 6 ARCA CP Echo-ARCA (9/27/00) Treadmill-nl (9/27/00) Age (y) Surgical Repair 11.0 Modified unroofing/ Pulmonary autograft 3 y postop 39.1 Modified unroofing and CABG (RIMA-RCA) 14.4 Modified unroofing 65.2 Modified unroofing 10.9 Modified unroofing 7.2 Modified unroofing 7 ALMCA CP/syncope Echo-ALMCA (4/2/01) Cath-ALMCA (4/2/01) 19.1 Neo-ostial creation without unroofing 8 ALMCA Exertional Echo-ALMCA (1/30/01) 11.4 Neo-ostial creation Dyspnea without unroofing 9 ARCA none Echo-ARCA (10/20/00) Perfusion scan-nl (11/13/00) Cath-ARCA (1/2/01) 10.6 Modified unroofing F/U Time (mo) Transthoracic Echocardiogram Exercise Study/Time Stress Echo 85 nl LCA origin/color flow, low-nl LV, mild AI 69 nl LCA origin, nl LV 4 nl LCA origin/color flow, nl LV 42 poor images, nl LV 17 nl RCA origin/color flow, nl LV 15 nl RCA origin/color Flow, nl LV 13 nl LCA origin nl LV 10 LCA dual ostia/ color flow, nl LV 9 nl RCA origin/color flow, nl LV 12.0 min 4.8 min, hypertensive response 11.2 min Upright bicycle: Q waves at baseline, no ST changes 10.9 min 13.0 min 12.0 min 13.8 min 13.2 min No ischemia, paradoxical septal motion present at rest and worsened with exercise No ischemia, diastolic relaxation abnl Not enrolled No ischemia, nl, limited windows, 4 apical segements not seen No ischemia, nl No ischemia, nl No ischemia, nl No ischemia, nl No ischemia, nl ALMCA anomalous origin of the left main coronary artery; ARCA anomalous origin of the right coronary artery; Cath cardiac catheterization; CP chest pain; Dx diagnosis; Echo echocardiogram; LCA left coronary artery; LV left ventricle; nl normal; RCA right coronary artery Ann Thorac Surg ROMP ET AL 2003;76: OUTCOME OF UNROOFING PROCEDURE 591

4 592 ROMP ET AL Ann Thorac Surg OUTCOME OF UNROOFING PROCEDURE 2003;76: Graded Exercise Testing Graded exercise testing with a Bruce protocol was performed in 8 of the patients. The 9th patient had Parkinson s disease, which precluded treadmill exercise; but that patient was able to perform graded exercise on an upright stationary bicycle. All tests were judged to be adequate based on achievement of a target heart rate of more than 85% of the age-based maximum heart rate. The mean duration of exercise in those performing treadmill testing was 11.4 minutes (median 12.0 minutes, range 4.8 to 13.8 minutes). Heart rate and blood pressure responses were appropriate in 8 patients. The 9th patient, a 39-year-old man with a history of hypertension, had an appropriate increase in heart rate but developed systolic hypertension with a systolic pressure of 228 mm Hg during maximal exertion. None of the 9 patients developed ST segment abnormalities or symptoms suggestive of myocardial ischemia during exercise testing. One 65-year-old patient had Q-waves at rest in the anterolateral leads suggestive of a prior anteroseptal infarction, but developed no pathologic changes with exertion. No patient developed ventricular arrhythmias during the course of exertion. In all cases, the indication to stop exercise was achievement of target heart rate or fatigue. Fig 1. The unroofing, as described by Mustafa and colleagues [11]. This was performed in 7 of 9 patients. In 2 patients the intramural segment was distal to the commissure and was unroofed without manipulation of the commissure. stenosis with a right internal mammary artery bypass graft. All 9 patients survived the operation. None experienced any significant perioperative complications. Of the 9 patients, 8 are free of reoperation or intervention. One patient (aged 11 years) underwent an unroofing of ALMCA with takedown and resuspension of the intercoronary commissure. He subsequently developed severe aortic insufficiency that had not been present on 4-month follow-up echocardiography. This necessitated aortic valve replacement with pulmonary autograft 44 months after the initial. Transthoracic Echocardiography Transthoracic echocardiography using two-dimensional imaging and color flow mapping was performed in all 9 surgical patients. In 8 of the 9 patients, the newly created coronary artery ostium was imaged in the correct sinus of Valsalva, and in 6 of the patients ostial patency was demonstrated by color flow mapping showing flow in the proximal coronary artery (Figs 3, 4). One of the 9 patients had inadequate images to visualize the coronary ostium by two-dimensional or color flow mapping. In all 9 patients, there was normal left ventricular function. The only patient with any significant aortic valvular insufficiency is the patient that subsequently required aortic valve replacement with the Ross. Fig 2. Unroofing performed on 2 of the 9 patients in whom a neo-ostium was created without taking down the intercoronary commissure. The new ostium is created by passing an instrument from the native ostium into the appropriate sinus. A neo-ostium is created in that sinus at the point at which the artery leaves the aortic wall.

5 Ann Thorac Surg ROMP ET AL 2003;76: OUTCOME OF UNROOFING PROCEDURE 593 Stress Echocardiography Eight patients underwent stress echocardiography at the time of exercise testing. The mean time of follow-up with stress echocardiography was 32.5 months (median 17.0 months, range follow-up 9 to 85 months). Images of the left ventricle were excellent and all 16 ventricular segments could be evaluated in 7 of 8 patients. In the 8th patient, the of the apical segments could not be visualized adequately to assess. Seven of the 8 patients had normal left ventricular. One patient, who was known to have pulmonary stenosis after the Ross, was noted to have flattening of the interventricular septal wall at rest that worsened with exertion, consistent with elevated right ventricular pressure. One patient, the 39-year-old man with a history of hypertension and atherosclerotic coronary artery disease, was believed to have diastolic relaxation abnormalities by stress echocardiography but no abnormalities of ventricular. No patients were found to have wall motion abnormalities suggestive of ischemic changes with exercise. Several other tests had been performed in the patients outside of protocol. One patient underwent rest and exercise thallium perfusion scanning 4 months after coronary artery repair. No perfusion defects were present at rest or with exercise. Two patients had cardiac catheterizations with coronary angiography performed subsequent to surgical repair of AAOCA. Patient 1 had catheterizations performed at 4 months, 42 months, and 80 months after repair. The first catheterization was for surveillance and showed an apparently normal coronary ostium and no significant aortic insufficiency. This patient subsequently developed aortic insufficiency, and a second catheterization was performed 42 months after initial surgery to quantify severity. There was no evidence of stenosis at the repaired coronary artery ostium at that time. This patient underwent a third catheterization 80 months after his coronary repair (35 months after pulmonary autograft ) for pulmonary valvuloplasty. No abnormality of the neo-coronary orifice was demonstrated on any of the catheterizations. A second patient had a catheterization performed before clearance for competitive athletics 5 months after repair and no abnormality of the coronary orifice was evident. Fig 3. Two-dimensional echocardiographic image of unrepaired anomalous left main coronary artery (ALMCA). There is a clear intramural segment with a common orifice with the right coronary artery and a slit-like opening. (AoV aortic valve; ICC intercoronary commissure; PA pulmonary artery.) Comment Anomalous aortic origin of a coronary artery is a rare and potentially lethal congenital anomaly. Increased risk of exertional sudden death is well described with both ALMCA and ARCA [2, 3, 12]. The combined incidences of these defects are reported to be between 0.1% and 0.3%. A common presentation is sudden unexplained death; however, angina, syncope, and arrhythmia can also be the presenting signs. The actual prevalence of this anomaly is impossible to know. Perhaps, because of increased awareness, more patients are presenting with symptoms suggestive of ischemia and are being diagnosed incidentally at catheterization or echocardiography [6, 10]. Autopsy series have shown that whereas some patients who die exhibit myocardial ischemia, others have no evidence of ischemic injury and may have experienced a lethal arrhythmia [13]. The pathophysiology of compromised coronary blood flow has many possible mechanisms. These include: (1) compression of the intramural segment of the coronary artery between the pulmonary artery and aorta; (2) narrowing of the slit-like orifice to Fig 4. Two-dimensional echocardiographic image of a patient who underwent operative repair with creation of a neo-ostium in the left coronary sinus. Image shows both native and neo-ostium. (ALMCA anomalous left main coronary artery.)

6 594 ROMP ET AL Ann Thorac Surg OUTCOME OF UNROOFING PROCEDURE 2003;76: this anomalous artery with increased aortic pressure; (3) an acute angle of the takeoff of the coronary artery; and (4) stenosis in the intramural segment of the coronary artery, especially at the level of the commissure. In an autopsy study, Taylor and associates [13] examined 30 cases of ALMCA and ARCA. Of these 30 individuals, 12 died after exertion. The authors found no relationship between the size or angle of the slit-like orifice and the risk of sudden death. Thus, risk stratification for this defect and prediction of which patients will benefit most from repair may not be possible. Hence, most recommend repair at diagnosis, regardless of the presence or absence of symptoms. Many surgical strategies have been suggested to treat this defect including coronary reimplantation, unroofing the intramural segment, and coronary artery bypass grafting. Coronary excision and reimplantation have been proposed. However, neo-ostial stenosis can occur and may result in the need for coronary artery bypass grafting. Coronary artery bypass grafting eliminates the need to open the aorta and manipulate the intercoronary commissure, but it subjects the patient to a bypass graft and the potential need for reintervention. Also, because the flow through the anomalous coronary artery is likely to be unobstructed at rest, an internal mammary bypass graft may have decreased patency because of competitive flow. This has led some authors to recommend ligation of the coronary artery proximal to the insertion of the graft [14]. Although this may be an acceptable therapy in an elderly patient with coexistent coronary artery disease, it seems imprudent in a young patient. The unroofing reported by Mustafa and colleagues [11] has been adopted by many surgeons with good immediate results. However, this approach often necessitates takedown and reattachment of the intercoronary commissure. This may affect aortic valve competency. Also, the long-term outcome of the neo-ostium has never been reported. In the current series, 1 of the patients subsequently underwent aortic valve replacement for development of severe aortic insufficiency. This did not occur immediately and was not present on an echocardiogram 4 months after initial surgery. He subsequently became symptomatic approximately 3 years after the initial and underwent aortic valve replacement with a pulmonary autograft operation. At the time of this operation the pathology noted was prolapse of the intercoronary commissure. Ideally, a new ostium of the coronary artery could be created in the appropriate sinus without disturbing the intercoronary commissure as shown in Figure 2. A similar technique has been reported in a patient with an anomalous left main coronary artery that arose from a separate orifice in the right sinus, immediately adjacent to the intercoronary commissure [15]. In that case, the surgeons chose to close the native orifice and to create a new orifice in the left sinus. In the 2 patients in our current series in whom a new ostium was created without unroofing or manipulation of the commissure, the postoperative echocardiogram demonstrated the new orifice in the appropriate sinus with a patent intramural segment. In both of these patients, the left coronary artery shared a common orifice with the right coronary artery in the right sinus of Valsalva. Consequently, the anomalous intramural left main artery was left patent for fear of disturbing the right coronary orifice. Few data exist regarding either the long-term patency of the new orifice of the coronary artery or the long-term relief of symptoms and risk of cardiac ischemia. In this study we used three accepted methods to assess the morphology of the aorta and coronary artery orifices as well as the presence of both rest and exercise-induced ischemia. Of the 8 patients that remained intervention free, 7 were symptomatic before surgery and all 7 were free of ischemic symptoms at follow-up. On prospective evaluation by transthoracic echocardiography, 6 of 7 patients who had undergone the unroofing were free of any aortic insufficiency. However, 1 of 7 patients (14%) did develop significant aortic insufficiency and underwent aortic valve replacement. It is likely that this complication is the result of the initial unroofing operation as well as the manipulation of the intercoronary commissure. The coronary artery orifices and initial proximal path can usually be visualized by transthoracic echocardiography. This is especially true in children. In 8 of 9 patients the orifices of the coronary arteries were well visualized. To assess functional outcome, we chose to perform both exercise stress testing and stress echocardiography. The results of these tests seem to be reassuring in that no patient had signs of ischemia with exercise by symptoms, electrocardiography, or echocardiography. However, in a series of athletes who had sudden death, nine of 27 had a normal echocardiogram and 6 of 27 had a negative exercise stress test a mean of 10 months before death [16]. Thus, there is clearly some limitation to the conclusions that can be drawn from the results of these tests. It is encouraging, however, that this potentially lethal defect can be repaired by a relatively simple operation with seemingly durable results. In conclusion, anomalous aortic origin of the coronary artery is a rare and potentially lethal anomaly. Because of the risk of sudden death caused by myocardial ischemia, surgical correction is usually indicated regardless of the presence of symptoms. Unroofing of the anomalous coronary artery with creation of a new ostium in the appropriate sinus is a safe and durable method to repair this defect, but there is some potential risk of disruption of the aortic valve apparatus. Neo-ostial creation without taking down of the intercoronary commissure may relieve the risk of ischemia without manipulation of the commissure. References 1. Benson PA, Lack AR. Anomalous aortic origin of left coronary artery. Arch Pathol 1968;86: Benson PA. Anomalous aortic origin of coronary artery with sudden death. Case report and review. Am Heart J 1970;79: Cheitlin MD, DeCastro CM, McAllister HA. Sudden death as the complication of anomalous left coronary origin from the anterior sinus of Valsalva. Circulation 1974;50:780 7.

7 Ann Thorac Surg ROMP ET AL 2003;76: OUTCOME OF UNROOFING PROCEDURE Alexander RW, Griffith GC. Anomalies of the coronary arteries and their clinical significance. Circulation 1956;14: Topaz O, DeMarchena EJ, Perin E, et al. Anomalous coronary arteries: angiographic findings in 80 patients. Int J Cardiol 1992;34: Yamanaka O, Hobbs RE. Coronary artery anomalies in 126,595 patients undergoing coronary arteriography. Cathet Cardiovasc Diagn 1990;21: 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 1998;62: Maron BJ, Shirani J, Poliac LC, et al. Sudden death in young competitive athletes: clinical, demographic and pathological profiles. JAMA 1996;276: Taylor AJ, Rogan KM, Virmani R. Sudden cardiac death associated with isolated congenital coronary artery anomalies. J Am Coll Cardiol 1992;20: Davis JA, Cecchin F, Jones TK, et al. Major coronary artery anomalies in a pediatric population: incidence and clinical importance. J Am Coll Cardiol 2001;37: Mustafa I, Gula G, Radley-Smith R, et al. Anomalous origin of the left coronary artery from the anterior aortic sinus: a potential cause of sudden death. Anatomic characterization and surgical treatment. J Thorac Cardiovasc Surg 1981;82: Liberthson RR, Dinsmore RE, Bharati S, et al. Abberant coronary artery origin from the aorta. Circulation 1974;50: Taylor AJ, Byers JP, Cheitlin MD, et al. Anomalous right or left coronary artery from the contralateral coronary sinus: high-risk abnormalities in the initial coronary artery course and heterogeneous clinical outcomes. Am Heart J 1997;133: Shah AS, Milano CA, Lucke JP. Anomalous origin of the right coronary artery from the left coronary sinus: case report and review of surgical treatments. Cardiovasc Surg 2000;8: van Son JA, Mohr FW. Modified unroofing in anomalous aortic origin of left or right coronary artery. Ann Thorac Surg 1997;64: Basso C, Maron BJ, Corrado D, et al. Clinical profile of congenital coronary artery anomalies with origin from the wrong coronary sinus leading to sudden death in young competitive athletes. J Am Coll Cardiol 2000;35: DISCUSSION DR CARL BACKER (Chicago, IL): Jim, that was a great presentation. I would add, in addition, a very timely presentation. This particular diagnosis seems to be popping up more and more often with the increasing expertise of our echocardiographers. They are looking for it in patients who present with other issues and are finding these now in asymptomatic patients. I have two questions for you. The first is what would you recommend for an asymptomatic patient with anomalous aortic origin of the coronary artery who has a normal workup: normal Holter, normal stress test, normal stress echo, normal nuclear scan, etc. In other words, every test that you can do turns out to be normal; what would you recommend for that patient? The second is if you could comment a little bit on the role of internal thoracic artery grafting. I noticed you did that in 1 patient. Dr Mavroudis and I have had a couple of patients in whom we have done both the unroofing and an ITA bypass as a backup. There are other series in which they have reported using only the internal thoracic artery as the method of surgical treatment. Could you comment on that? Again, that was a great presentation. This is going to be very useful to many of us. Thank you. DR JAGGERS: Thank you, Dr Backer. The situation described is actually one that is not that uncommon. In a series of athletes generated between Seattle and Italy, in a nice series of autopsy patients (of course, all these patients died), when they looked back to find out which patients were symptomatic, there were 12 patients that were symptomatic. Of those patients, 9 had had some preoperative evaluation and 6 of those patients had had preoperative exercise treadmill testing; in all 6 of those patients, the exercise treadmill testing was negative. So I think in that particular situation, if we have an anatomic defect that we know has a sudden risk, I think it probably behooves us to go ahead and repair that despite the presence or absence of a negative workup. DR SCOTT BRADLEY (Charleston, SC): In all of your patients, did the anomalous coronary run between the aorta and the pulmonary artery? DR JAGGERS: That s correct. DR BRADLEY: In that situation, have you seen the coronary to have a course which is not intramural? If that occurs, have you been able to successfully figure it out preoperatively, and does that affect your ability to perform the operation you have described? DR JAGGERS: I think that is an important point. There are a couple of case reports talking about a coronary artery that arises from the wrong sinus and travels intact between the pulmonary artery and the aorta. There was a nice case report presented by Rodafeld and colleagues in which this was a well-formed coronary artery that traveled between the aorta and the pulmonary artery. In that situation I think it is probably not a good idea to unroof this because you are essentially creating the part of the wall that is not aorta; it is just coronary artery. In that situation they performed a pulmonary artery translocation in which they just simply reimplanted the pulmonary artery distally into the left pulmonary artery, hence, pulling the pulmonary artery and the aorta apart. that would be one approach to that. So I would approach that patient differently. Echocardiography should be good enough to tell that difference, if you have good echocardiographers. And to address the internal mammary issue that Dr Backer brought up, the internal mammary artery that we did was a right internal mammary artery in a 39-year-old patient who had coexistent proximal right coronary artery disease, and we still unroofed the anomalous left coronary artery but did a right internal mammary artery to the right coronary artery. I think coronary artery bypass grafting is an option. However, if one does a coronary artery bypass graft and leaves the slit-like orifice in a patient, most of the time the coronary blood flow to this artery is normal. Therefore, you have got competitive flow through your mammary graft, leading some authors to propose tying off that proximal coronary artery, which I think subjects a young patient to introgenic coronary artery disease. DR CONSTANTINE MAVROUDIS (Chicago, IL): John Brown from Indianapolis is in the audience today and perhaps he

8 596 ROMP ET AL Ann Thorac Surg OUTCOME OF UNROOFING PROCEDURE 2003;76: would like to comment on this very interesting disease process. Doctor Jaggers is operating on patients who are asymptomatic and for all intents and purposes have a normal course of a coronary artery except a little compression. Not only that, but he caused 1 patient to have a Ross operation. So what do you think about that? And then what do you think about this competitive flow in the coronary artery? Frankly, this is a difficult problem and I m not sure that competitive flow plays a big part in this disease. What about you? DR JOHN W. BROWN (Indianapolis, IN): Well, I m afraid I have been on both sides of the fence. I have done the internal mammary graft to an artery that I have unroofed because I wasn t as confident about my repair as maybe I should have been. It is really a fairly straightforward operation. Most of the time you can get by, or at least I have been able to get by without disturbing the aortic commissure, which would bother me. I think the technique that you described was quite a novel technique. I wasn t clear. I understood that in the neo-ostia that you performed, there was actually flow through the original ostia, the native ostia, and the neo-ostia. Why would there have been both? And the commissures seemed to be sort of stretched between the two. Do you think you should have probably closed the original ostia and that would have supported your aortic commissure better and maybe have avoided the need for the Ross down the road? DR JAGGERS: In 2 of the patients in whom I did not manipulate the commissure, simply passed a clamp between here, there will be flow going in this intramural segment; however, with exercise it is unlikely that flow will come through this intramural segment because it is getting compressed. In these particular patients, the ostia was shared with the right coronary artery, and I didn t want to manipulate that ostia for fear that I might compromise right coronary flow in some way. So I simply created a new ostia, and I am trusting that the antegrade flow through this new ostia will be more efficient than flow through this intramural segment. However, the echocardiogram that I showed, this is an immediate postoperative echocardiogram in which there is still flow. In 1 of the 2 patients that I did this on at 29 months, there was no flow within this intramural segment. DR BROWN: Gus, the other thing, I think in the asymptomatic patient, the trouble is the sudden death incidence is high enough to make me want to go ahead and operate on most of those patients even though they are asymptomatic.