Coronary Artery from the Wrong Sinus of Valsalva: A Physiologic Repair Strategy Tom R. Karl, MS, MD he most commonly reported coronary artery malformation leading to sudden death in children and young ath-nocent murmur investigation and evaluation of performance amination of their proximal coronary anatomy as part of in- T letes is an anomalous coronary origin from the wrong aorticstatus, four patients (0.2%) were found to have an anomalous sinus, with or without a proximal course of the coronary origin of the coronary artery from the wrong sinus. 2 artery between the aorta and the pulmonary artery. Although Whatever the true prevalence, this lesion predisposes to the incidence is difficult to assess, there is a 0.17% incidencefatal episodes of myocardial ischemia that may occur during in autopsy series (all variants included) and a 0.1 to 0.3% inor shortly after strenuous exercise. 3-5 Timely diagnosis requires a high index of suspicion but is frequently impossible, catheter-echo diagnostic series. There may be a regional variation. In a prospective study of 1950 consecutive patients as the majority of patients with the anomaly are asymptomatic and thus do not seek medical attention. Therefore, undergoing coronary angiography, anomalous origin of the right coronary artery from the left aortic sinus was found in sudden death is frequently the first manifestation, especially 0.92% of cases, whereas the incidence of left coronary artery in young athletes, with only 30% of patients reporting prodromal symptoms. arising from the right aortic sinus was 0.15%. 1 In a study of 2388 patients who had transthoracic echocardiographic ex- 5,6 Screening young adults before participation in competitive sports has been unsuccessful. Stress testing is unreliable because of high false-positive and falsenegative results; echocardiogram, echocardiography, and Division of Pediatric Cardiothoracic Surgery, University of California San Francisco School of Medicine, San Francisco, California. Address reprint requests to Tom R. Karl, MS, MD, Professor of Surgery, UCSF School of Medicine, Room S-549, 513 Parnassus Avenue, San Francisco, CA 84143. E-mail: karlt@surgery.ucsf.edu stress testing all lack predictive value. 4,5 Finally, although the major association of sudden death is with young athletes, cases have also been reported in children under 1 year of age. 1522-2942/08/$-see front matter 2008 Elsevier Inc. All rights reserved. doi:10.1053/j.optechstcvs.2008.01.001 35
36 T.R. Karl Operative Technique Figure 1 Sudden death from myocardial ischemia has been observed most commonly with the left coronary artery (LCA) from the right sinus but is also seen in the right coronary artery (RCA) from the left sinus, and LCA from the noncoronary sinus. 7 Several mechanisms have been proposed to explain the pathophysiology of acute myocardial ischemia with anomalous coronary artery origin from the wrong sinus, including the following: (1) flap closure of the slit-like opening of the coronary orifice; (2) acute (nonorthogonal) branching angle and kinking of the coronary artery as it exits the aorta; (3) intramural segment of the proximal coronary artery; (4) compression of the intramural segment by the aortic commissure; (5) compression of the coronary artery as it courses between the aorta and the pulmonary artery, accentuated by exercise-related expansion of the pulmonary artery; (6) spasm of the coronary artery as the result of endothelial injury. 1,2 The only effective treatment for this problem is surgery, and various techniques have been described, each having a role in selected cases. 8-10 We present herein a universal surgical strategy that could be applied for all variants of this disease, irrespective of coronary ostial configuration, proximity of aortic valve, or other features. There is no risk to the aortic commissures or valve leaflets, and all of the pathophysiological mechanisms, as we understand them, may be addressed. Ao aorta; L left; MPA main pulmonary artery; N non; R right.
Coronary artery from the wrong Sinus of Valsalva 37 Figure 2 Repair is performed through a median sternotomy. An autologous pericardial patch is procured during opening and fixed in 0.2% glutaraldehyde for 5 minutes, followed by a 10-minute saline rinse. The patient is placed on cardiopulmonary bypass using aortic and bicaval cannulation and a left ventricular vent and cooled to 32 C. The aorta is clamped and the heart is arrested with aortic root cold blood cardioplegia, or with a combination of antegrade and retrograde cardioplegia in cases known to have ostial stenosis. The aorta is transected. Beginning from the cut edge of the aorta, an incision is made into the ostium of the anomalous coronary and extended into the coronary itself for about 1 cm, either stopping short of the bifurcation or continuing into the larger branch. A triangular patch of the glutaraldehyde-treated autologous pericardium is sutured into this incision to enlarge the diameter of the proximal coronary artery trunk, creating an ostium of about 5 mm. 11 The aortic anastomosis is completed, incorporating the base of the triangular pericardial patch into the aortic anastomotic suture line. The heart is then deaired, and the cross-clamp is removed. L left; LCA left coronary artery; R right; RCA right coronary artery.
38 T.R. Karl Figure 3 In patients in whom the anomalous coronary courses between the aorta and the pulmonary artery, the main pulmonary artery is transected just proximally to its bifurcation. The incision is carried into the left pulmonary artery branch. The main pulmonary artery is anastomosed to the left pulmonary artery. The defects at the bifurcation and in the origin of the right pulmonary artery are closed with a second pericardial patch to avoid stenosis. The goal of this translocation is to move the pulmonary artery away from the aorta, thereby reducing the chance for compression of the anomalous coronary artery as it courses between the aorta and pulmonary artery. 10 The patient is warmed fully and weaned from cardiopulmonary bypass, usually with good contractility of both ventricles, unless ischemic injury has occurred before operation. Ao aorta; LCA left coronary artery; MPA main pulmonary artery.
Coronary artery from the wrong Sinus of Valsalva 39 Comment Our technique enlarges the slit-like ostium and augments the diameter of the proximal coronary, while improving the acute angulation at takeoff. The technique can be used even when there is a common origin of the two coronary arteries. Finally, the pulmonary artery translocation increases the anatomic space between the arterial trunks and eliminates the risk of coronary artery compression. We have used this technique in seven patients (age 2 years to 52 years). One patient, who presented in cardiogenic shock, required temporary left ventricular assist device support postoperatively. All patients are symptom free at a mean follow-up interval of 29 months (range, 4 to 85 months). Laminar flow through the new ostium was seen with color Doppler or two-dimensional echo in all cases examined (6/7) and all patients in this study group have had normal echocardiogram stress echo, with no wall motion defects. There have been no reoperations nor late deaths, with all patients enjoying unrestricted activities. 7 Conclusions In conclusion, in cases of anomalous coronary artery from the wrong aortic sinus, with a proximal course between the aorta and the pulmonary artery, the combination of coronary artery angioplasty and translocation of the pulmonary artery represents a physiologic repair strategy that effectively addresses all the mechanisms that can generate ischemia. The technique is simple and effective and has shown good midterm results. References 1. Angelini P: Coronary artery anomalies current clinical issues: definitions, classification, incidence, clinical relevance, and treatment guidelines. Tex Heart Inst J 29:271-278, 2002 2. Davis JA, Cecchin F, Jones TK, et al: Major coronary artery anomalies in a pediatric population: incidence and clinical importance. J Am Coll Cardiol 37:593-597, 2001 3. Basso C, Corrado D, Thiene G: Congenital coronary artery anomalies as an important cause of sudden death in the young. Cardiol Rev 9:312-317, 2001 4. Basso C, Maron BJ, Corrado D, et al: 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 35:1493-1501, 2000 5. Liberthson RR: Sudden death from cardiac causes in children and young adults. N Engl J Med 334:1039-1044, 1996 6. Maron BJ, Shirani J, Poliac LC, et al: Sudden death in young competitive athletes: clinical, demographic, and pathological profiles. JAMA 276: 199-204, 1996 7. Alphonso N, Anagnostopoulos PV, Nölke L, et al: Anomalous coronary artery from the wrong sinus of Valsalva: a physiologic repair strategy. Ann Thorac Surg 83:1472-1476, 2007 8. Ono M, Brown DA, Wolf RK: Two cases of anomalous origin of LAD from right coronary artery requiring coronary artery bypass. Cardiovasc Surg 11:90-92, 2003 9. Garcia-Rinaldi R: Right coronary arteries that course between aorta and pulmonary artery. Ann Thorac Surg 74:973-974, 2002 10. Rodefeld MD, Culbertson CB, Rosenfeld HM, et al: Pulmonary artery translocation: a surgical option for complex anomalous coronary artery anatomy. Ann Thorac Surg 72:2150-2152, 2001 11. Patel K, Davidson A, Karl TR: Anomalous left coronary artery arising from the right coronary cusp. Ann Thorac Surg 71:2045, 2001