Ann Thorac Surg 2007;83:1472-1476
© 2007 The Society of Thoracic Surgeons
Original Articles: Cardiovascular
Anomalous Coronary Artery From the Wrong Sinus of Valsalva: A Physiologic Repair Strategy
Nelson Alphonso, MD,
Petros V. Anagnostopoulos, MD,
Lars Nölke, MD,
Anita Moon-Grady, MD,
Anthony Azakie, MD,
Gary W. Raff, MD,
Tom R. Karl, MD*
Pediatric Heart Center, UCSF Children’s Hospital, University of California San Francisco, San Francisco, California
Accepted for publication October 24, 2006.
* Address correspondence to Dr Karl, UCSF Division of Pediatric Cardiothoracic Surgery, 513 Parnassus Ave, Suite S-549, Box 0117, San Francisco, CA 94143-0117 (Email: karlt{at}surgery.ucsf.edu).
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Abstract
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Background: A coronary artery arising from the wrong aortic sinus, with or without a proximal course between the aorta and the pulmonary artery, may predispose to myocardial ischemia, myocardial infarction, or sudden death.
Methods: We repaired this lesion in 5 symptomatic patients, all of whom had experienced exercise-related chest pain, dyspnea, or syncopal episodes. All patients underwent patch angioplasty of the proximal anomalous coronary artery with glutaraldehyde-treated autologous pericardium. When the coronary artery coursed between the aorta and the main pulmonary artery, the main pulmonary artery was translocated toward the left pulmonary artery to create additional space and prevent compression.
Results: All patients recovered well and to date have demonstrated no evidence of recurrent myocardial ischemia.
Conclusions: The combination of coronary artery angioplasty and translocation of the pulmonary artery seems to effectively address all the mechanisms that can generate ischemia.
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Introduction
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Various abnormalities in the cardiovascular system have been implicated in sudden death among children and young adults. Among the most consistent lesions leading to these rare, but widely publicized deaths are a variety of coronary artery anomalies. The most commonly reported coronary artery malformation in sudden death series, both in the child and in the young athlete, is an anomalous origin from the wrong aortic sinus, with or without a proximal course of the artery between the aorta and the pulmonary artery. This lesion predisposes to fatal episodes of myocardial ischemia that may occur during or shortly after strenuous exercise [1–3]. Timely diagnosis requires a high index of suspicion, but is frequently impossible, as the majority of patients with this anomaly are asymptomatic and thus do not seek medical attention. Sudden death is frequently the first manifestation, and only 30% of patients report prodromal symptoms, such as chest pain, shortness of breath, and dizziness [3, 4]. The only effective treatment for this problem is surgical. Various techniques have been described to deal with this malformation [5–7].
In this article, we review our experience and describe our strategy for repair of anomalous coronary artery originating from the wrong aortic sinus, with or without a proximal course between the aorta and the pulmonary artery.
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Patients and Methods
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Approval for this study was obtained from our Institutional Review Board, and individual patient consent was waived. The patients, methods, and outcome are summarized in Table 1.
Patients
Patient 1
A 11-year-old girl had a syncopal episode after moderate exercise. She was found to have ventricular tachycardia and was cardioverted by paramedics at the scene. The arrhythmia was thought to be primary, and she underwent an electrophysiologic study. Upon induction of anesthesia, she had signs of diffuse myocardial ischemia. Cardiac catheterization was performed, revealing an anomalous origin of the left coronary artery from the noncoronary sinus, with no flow through the vessel. An intra-aortic balloon was placed for stabilization, and she was transferred to the operating room. At operation, we found an anomalous origin of the left coronary artery from what would have been the noncoronary sinus, with no coronary orifice in the anatomic left sinus. The orifice was slitlike, with an acute angle of exit from the aorta. The artery coursed posterior to the pulmonary artery, and did not course between the aorta and the pulmonary artery.
Patient 2
A 14-year-old boy had three syncopal episodes that occurred after strenuous exercise. During the last episode, blood tests showed elevated serum troponin levels. Echocardiographic examination revealed an anomalous origin of the left main coronary artery from the right aortic sinus, with a proximal course between the aorta and the pulmonary artery. Results of cardiac catheterization were suspicious for a slitlike orifice of the anomalous coronary artery. At operation, the left coronary artery was found arising from the anatomic right aortic sinus, close to the commissure between the left and right aortic valve cusps. The orifice was slitlike with a proximal intramural segment. The artery subsequently coursed between the aorta and the pulmonary artery.
Patient 3
A 10-year-old boy had a syncopal episode while playing at school. An echocardiogram was nondiagnostic. Cardiac catheterization demonstrated an origin of the right coronary artery from the anatomic left coronary sinus, with a slitlike appearance of the ostium. The anomalous coronary artery coursed between the aorta and the pulmonary artery. At operation, there was a single coronary artery arising from the left coronary sinus of Valsalva. The coronary ostium was normal. The right coronary artery arose from the single coronary artery and coursed between the aorta and the pulmonary artery.
Patient 4
A 14-year-old boy collapsed during basketball practice and was resuscitated by a bystander until the paramedics arrived at the scene. He was found to be in ventricular fibrillation and was promptly defibrillated. Blood tests revealed elevated serum creatine phoshokinase (myocardial fraction) and troponin levels. Echocardiography and cardiac catheterization showed mild global hypokinesis and a single coronary artery arising from the anatomic right sinus of Valsalva. The left coronary artery branched from the single coronary artery and coursed between the aorta and the pulmonary artery. There was angiographic and echocardiographic evidence of substantial systolic compression of the left coronary artery in this location. At operation, there was a common slitlike orifice located in the right aortic sinus. The initial segment of the left coronary was intramural. It exited from the aortic wall near the valve commissure between the anatomic right and left coronary cusps and coursed between the pulmonary artery and the aorta.
Patient 5
A 10-year-old boy presented with weekly episodes of exertional dyspnea and chest pain. He had a negative thallium stress test. Multiple serum troponin blood samples during the episodes of chest pain were negative for ischemia. Echocardiogram was suggestive of anomalous origin of the right coronary artery from the left coronary sinus with a course between the aorta and the pulmonary artery. The diagnosis was confirmed with magnetic resonance imaging. At operation, the orifice of the right coronary artery was slitlike and emerged from the left aortic sinus adjacent to the commissure between the left and right aortic valve cusps. The stenotic orifice would barely admit a 2-mm probe. The first part of right coronary artery was intramural. The artery then coursed between the aorta and the pulmonary artery.
Surgical Technique
All repairs were performed through median sternotomy. An autologous pericardial patch was harvested and fixed in glutaraldehyde. Patients were placed on cardiopulmonary bypass using aortic and bicaval cannulation, and cooled to 32°C. The aorta was clamped and the heart was arrested with antegrade cold blood cardioplegia. In the 1 patient who presented in extremis, a combination of antegrade and retrograde cardioplegia was used.
The aorta was transected. Beginning from the cut edge of the aorta, an incision was made into the ostium of the anomalous coronary. The incision was 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 was sutured into this incision to enlarge the diameter of the proximal coronary artery trunk, creating an ostium of about 5 mm [8] (Fig 1 A, B, C). The aortic anastomosis was then completed, incorporating the top edge of the triangular pericardial patch into the aortic anastomotic suture line (Fig 2 A). The heart was then deaired, and the cross clamp was removed.
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Fig 1. Technique for correction of left coronary artery from right sinus of Valsalva. (A) The aorta and pulmonary artery have been transected above the commissures. The eccentric slit orifice in the wrong sinus of Valsalva, proximal intramural segment, nonorthogonal angle of exit, and interarterial course of the left coronary artery are demonstrated. (B) The left coronary artery has been opened from its origin in the aorta to the bifurcation. (C) An autologous pericardial patch is sutured into the coronary artery and aortic wall.
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Fig 2. (A) The pulmonary artery is anastomosed in the anatomic position for cases without an interarterial course. (B) For cases with an interarterial course, the proximal pulmonary artery is translocated to the left pulmonary artery, with patch augmentation of the right pulmonary artery.
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In the 4 patients in whom the anomalous coronary coursed between the aorta and the pulmonary artery, the main pulmonary artery was transected just proximal to its bifurcation. The incision was then carried into the left pulmonary artery branch. The main pulmonary artery was then anastomosed to the left pulmonary artery. The defect at the bifurcation and in the origin of the right pulmonary artery was closed with a pericardial patch to avoid stenosis (Fig 2 B). The goal of this translocation was to move the pulmonary artery away from the aorta, thereby reducing the chance for compression of the anomalous coronary artery coursing between the aorta and pulmonary artery [7].
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Results
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There were no deaths. The first patient described herein was supported with a left ventricular assist device for 72 hours. Her myocardial function improved rapidly, and after weaning from the assist device, she had an uneventful hospital course. Two years later, she is asymptomatic with normal left ventricular function. The second patient recovered well from his operation. During his follow-up, he had a negative exercise treadmill test and a coronary angiogram that showed widely patent coronary arteries. He is currently asymptomatic 13 months after his repair. The third patient also remains asymptomatic. Four years after the repair he underwent an exercise treadmill test during which he displayed normal cardiac response to maximal exercise with no evidence of ischemia. The fourth patient is asymptomatic 18 months after his operation. At 6 months, he underwent an exercise treadmill test that was negative for ischemia, an exercise echocardiogram that was normal, an electrophysiologic study that did not show any inducible ventricular arrhythmia, and a cardiac catheterization that showed widely patent coronary arteries with no evidence of obstruction. At 1 year after surgery, he had an electrophysiologic study that showed couplets and triplets but no inducible ventricular tachycardia with programmed stimulation. The last patient also recovered uneventfully from the operation and is doing well 13 months after the operation.
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Comment
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How Prevalent is This Lesion?
Cheitlin and coworkers [9], in 1974, described clinical and autopsy findings in 13 patients in whom the left coronary artery arose from the wrong aortic sinus. They linked this anomaly to sudden death and called it a "not so minor congenital anomaly." The prevalence of anomalous coronary artery from the wrong aortic sinus is not known. In a prospective study of 1,950 consecutive patients undergoing coronary angiography, anomalous origin of the right coronary artery from the left aortic sinus was found in 0.92% of cases, whereas the incidence of left coronary artery arising from the right aortic sinus was 0.15% [10]. In a study of 2,388 patients who had transthoracic echocardiographic examination of their proximal coronary anatomy as part of innocent murmur investigation and evaluation of performance status, 4 patients (0.2%) were found to have an anomalous origin of the coronary artery from the wrong sinus [11]. The reported proportion of patients with anomalous origin of a coronary artery in these studies probably does not represent the true prevalence in an unselected population, because of a referral bias. If it did, there would be close to 3 million persons affected with this anomaly in the United States [10].
The Diagnostic Challenge
The diagnosis is often made postmortem, as most of the patients are asymptomatic, with vague cardiovascular symptoms occurring in 18% to 30% [3, 4, 11]. Screening young adults before participation in competitive sports has been unsuccessful. Stress testing is unreliable because of high false-positive and false-negative results [2, 3]. Although echocardiographers can identify an anomalous origin of a coronary artery, coronary anatomy is seldom the focus of examination in asymptomatic young athletic persons [2, 11]. Even in symptomatic patients, a correct diagnosis requires a very high index of clinical suspicion, as illustrated by the cases presented here, in which the coronary artery was only demonstrated to be clearly abnormal by echocardiography in 3 patients. The presentation falls into one of three categories: dyspnea, syncope, or aborted cardiac death. All our patients presented with one or more of these signs or symptoms. The fact that a patient with aborted cardiac death was first thought to have a primary ventricular arrhythmia rather than an anomalous coronary artery origin underlines the real challenge in diagnosing these lesions, even in units that have very experienced pediatric echocardiographers and electrophysiologists.
The Electrophysiologic Issue
Sudden death with anomalous origin of the coronary artery from the wrong aortic sinus results from episodes of myocardial ischemia that are cumulative with time [1, 3]. Pathology data show that in hearts of patients with this anomaly there is patchy myocardial necrosis and fibrosis from previous ischemic episodes that can create an unstable myocardial substrate and predispose to lethal ventricular tachyarrhythmias [1]. Surgical correction of the anomalous coronary artery origin does not address the pathologic changes discussed above. Therefore, one might speculate as to whether all these patients should undergo a preoperative electrophysiologic study to determine the need for an automatic implantable cardioverter defibrillator and, more importantly, whether the operation alone prevents sudden death. The need for regular electrophysiologic studies in the follow-up period also remains unclear. Only 1 patient in our series had postoperative electrophysiologic studies (the first of which was negative and the second equivocal). To date, no patients reported in the surgical literature (including our own) who had this lesion corrected had evidence of late postoperative ventricular arrhythmias. In addition, none of the patients reported has had syncopal episodes or died unexpectedly after the operation.
What Is the Optimal Surgical Strategy?
Several mechanisms have been proposed to explain the pathophysiology of acute myocardial ischemia with anomalous coronary artery origin from the wrong sinus [10, 11]: (1) flap closure of the slitlike opening of the coronary orifice; (2) acute angle of take-off and kinking of the coronary artery as it exits from 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; and (6) spasm of the coronary artery as the result of endothelial injury.
In patients with a proximal intramural segment, studies with intravascular ultrasonography have shown a thinning of the common aortocoronary wall [9]. This common wall is weak in the young and yields during the ejection period, resulting in luminal compression that is worse during systole. Normal stiffening of the aortic wall that occurs with aging may account for the benign course of the anomalous coronary origin from the wrong sinus in older patients [9].
To correct the slitlike opening of the anomalous coronary artery origin from the wrong aortic sinus, excision of the common wall of the aorta and the coronary artery has been proposed. By enlarging the coronary ostium, this operation eliminates the flap closure of the opening of the coronary orifice and compensates for the acute angulation of the anomalous coronary artery as it arises from the aorta [6]. This operation, although easy to perform, may not be suitable when the anomalous coronary artery shares a common ostium with the other coronary artery [7]. In addition, when the coronary ostium is eccentrically located in the aortic sinus, the repair of the ostium may require detachment of the commissure and can compromise the competence of the aortic valve. In autopsy studies, the shape of the coronary ostium alone was not helpful in predicting the clinical significance of this anomaly [12]. In cases where the anomaly was considered responsible for the death of the patient, the ostium was sometimes a slit and in others round [12]. Furthermore, this operation does not address the increased risk of sudden death due to compression of the coronary artery as it courses between the aorta and the pulmonary artery [2, 6].
Rodefeld and associates [6] described translocation of the main pulmonary artery to the left pulmonary artery branch with patch closure of the opening to the right pulmonary artery to address the potential for compression between the aorta and pulmonary artery. This reimplantation creates additional space between the aortic and pulmonary trunks [6] and has the advantage of not requiring an aortic root procedure. However, this technique alone does not correct the abnormalities of the anomalous coronary ostium. The coronary artery bypass graft, although described, has poor long-term patency in children, especially in the presence of unobstructed antegrade competitive flow [5, 6, 13]. Coronary reimplantation is another strategy that has been used to correct this problem [14]. This operation is technically demanding and may be problematic when there is a common origin of both right and left coronary arteries [6]. Furthermore, any of the options involving coronary transfer or unroofing have a potential for injury or distortion of the aortic valve [15].
The various techniques used in the past for wrong sinus origin of a coronary artery have worked well in properly selected cases, and probably all of them are useful today as well. Our strategy, however, is presented as a universal solution to the problem that could be applied for all variants of this disease, irrespective of coronary ostial configuration, proximity of aortic valve, or other features as discussed above. Our strategy also takes into account all the potential mechanisms that can generate ischemia in any given patient, which is not the case with previously described operations. We use an autologous pericardial patch to augment the ostium of the anomalous coronary artery, leaving the commissure of the aortic valve undisturbed. It enlarges a slitlike ostium and augments the diameter of the proximal coronary, while improving the acute angulation at the take-off. The technique can be used even when there is a common origin of the two coronary arteries. The reported midterm patency of proximal coronary artery arterioplasty with autologous patch material in children and adults has been satisfactory [16, 17]. Finally, the pulmonary artery translocation increases the anatomic space between the arterial trunks and eliminates the risk of coronary artery compression.
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.
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References
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Abstract
Introduction
