Ann Thorac Surg 2009;88:1339-1341. doi:10.1016/j.athoracsur.2009.02.089
© 2009 The Society of Thoracic Surgeons
Case Reports
Atypical Presentation of Anomalous Origin of the Left Main Coronary Artery From the Pulmonary Artery
Takeki Suzuki, MD, MPH*,
Frank P. Ittleman, MD,
Prospero B. Gogo, Jr, MD
Cardiology Division and Cardiothoracic Surgery Division, University of Vermont College of Medicine, Fletcher Allen Health Care, Burlington, Vermont
Accepted for publication February 20, 2009.
* Address correspondence to Dr Suzuki, McClure One, Cardiology Division, Fletcher Allen Health Care, 111 Colchester Ave, Burlington, VT 05401 (Email: suzukit_tky{at}hotmail.com).
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Abstract
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Anomalous origin of the left main coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly that usually presents in childhood. Ninety percent of the patients with ALCAPA die within the first year of life without surgical intervention. In adults, ALCAPA is associated with left ventricular dysfunction, mitral regurgitation, and sudden death. The present report describes the case of an adult patient who presented with an abnormal stress test and ALCAPA was diagnosed during cardiac catheterization.
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Introduction
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Anomalous origin of the left main coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly that usually presents in childhood. The first comprehensive clinical description of ALCAPA was published by Bland and colleagues [1] in 1933. We describe a patient who presented with an abnormal stress test and ALCAPA was diagnosed during cardiac catheterization.
A 50-year-old woman was referred to our institution for cardiac catheterization. Six months prior to presentation she began experiencing exertional chest tightness, located on her left side, without radiating pain to either her arm or jaw, and with resolution when resting. It was associated with dyspnea and palpitations. These symptoms had been stable for several months. She denied dizziness, diaphoresis, orthopnea, paroxysmal nocturnal dyspnea, leg swelling, nausea, or vomiting. Three days prior to cardiac catheterization, she underwent pharmacological stress perfusion imaging, which revealed a small anteroapical myocardial infarction in the left anterior descending artery territory and moderate peri-infarct ischemia. Left ventricular (LV) systolic function was normal.
On physical examination she was not in acute distress. Her systolic blood pressure was 124 and her diastolic blood pressure 74, which was equal in both arms. Her heart rate was 64 beats per minute. She was afebrile and saturating 99% on ambient air. There was no jugular venous distension. Lungs were clear to auscultation bilaterally. Heart examination showed regular rate and rhythm, with normal heart sounds, and without murmurs, rubs, or gallops. No lower extremity pitting edema was appreciated. Pulses were 2+ and they were symmetric in the upper and lower extremities.
Laboratory values were unremarkable. Electrocardiography showed normal sinus rhythm and left axis deviation. There were no ST segment deviations or any pathologic Q waves.
In the catheterization laboratory, attempts to engage the left coronary artery were unsuccessful. Angiography of the right coronary artery (Fig 1) showed an extraordinarily large vessel and an abundant collateralization to a similarly ectatic left coronary system. The entire left coronary system was perfused backward from the right coronary artery. There was reversal of flow into the left main coronary artery, which with further examination was found to originate from the main pulmonary artery. There was a focal 60% stenosis in the ostium of the left main coronary artery. Because of these findings, a right heart catheterization was performed. There was normal mean right atrial and right ventricular diastolic pressure, no evidence of pulmonary or right ventricular systolic hypertension, and no significant left-to-right shunting by multisite oximetric sampling. Echocardiography showed mitral regurgitation and normal LV systolic function without wall motion abnormalities.
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Fig 1. Angiography of the right coronary artery showed an extraordinarily large vessel (8 mm in diameter) and abundant collateralization to a similarly ectatic left coronary system. There was a focal 60% stenosis in the ostium of the left main coronary artery.
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Given the symptoms of the patient, as well as objective findings of ongoing injury and ischemia, revascularization of the anterior wall was indicated. Cardiothoracic surgery was consulted and she was brought to the operating room. Cardiopulmonary bypass was used. First, direct reimplantation of the left main artery to the aorta was considered, but was not possible because there was not enough length of the left coronary artery to reach the aorta. Second, reimplantation of the left main artery with a use of interposition graft was considered. However, because the left main coronary artery originated from the posterior wall of the pulmonary artery and there was a concern about kinking, this was not an option. Finally, she underwent obliteration of the origin of the left main coronary artery from the pulmonary artery and received left internal mammary artery grafting to the left anterior descending artery. The left internal mammary artery grafting to the left anterior descending artery was performed because the ischemia seen in the stress test was located in that territory. There was no evidence of atherosclerosis at all in the focal stenosis of the ostium of the left main coronary artery. Postoperatively, she had been doing well and was discharged home without any complication. She has been followed-up and her previous symptoms disappeared after the surgery.
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Comment
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We present a case with ALCAPA. This rare anomaly (ie, ALCAPA), also known as Bland-White-Garland syndrome, usually presents in childhood. The incidence of ALCAPA is 1 in 300,000 live births [2]. Ninety percent of the patients with ALCAPA die within the first year of life without surgical intervention.
The prevalence in adults is reported to be 1 to 5 in 10,000, identified by coronary angiography [3]. In adults, ALCAPA is associated with LV dysfunction, mitral regurgitation, and sudden death. Some of the patients have an ostial stenosis in the left main coronary artery [4]. This patient presented in her late 50s and a focal stenosis in ostium of the left main coronary artery likely prevented her from significant steal syndrome for most of her life.
Surgery and percutaneous closure are treatment options. Surgical techniques include: (1) ligation of the left main artery with revascularizaton of the left coronary system using vein graft or internal mammary artery grafting, and (2) reimplantation of the left main artery to the aorta directly or indirectly through a pulmonary artery tunnel or through an internal iliac artery graft [5]. Ligation of the left main artery was performed in 1959 [6]. This technique was shown to higher early and late mortality rates [7]. The high mortality was believed to be due to the fact that postoperatively the patients have one-vessel system. Subsequently, ligation of the left main artery with revascularization of the left coronary system was introduced and has been successful. Reimplantation of the left main artery to the aorta may not be possible because of anatomical reasons. Ligation of the anomalous artery with coronary artery bypass grafting has been the preferred approach in adults, and reimplantation of the left main coronary artery into the aorta has been the most frequently used surgical technique in the pediatric population [7]. Percutaneous ALCAPA closure using an Amplatzer Vascular Occluder has been recently reported [8].
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References
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- Bland EF, White PD, Garland J. Congenital anomalies of the coronary arteries: report of an unusual case associated with cardiac hypertrophy Am Heart J 1933;8:787-801.
- Keith JD. The anomalous origin of the left coronary artery from the pulmonary artery Br Heart J 1959;21:149-161.[Free Full Text]
- Wesselhoeft H, Fawcett JS, Johnson AL. Anomalous origin of the left coronary artery from the pulmonary trunk. Its clinical spectrum, pathology, and pathophysiology, based on a review of 140 cases with seven further cases. Circulation 1968;38:403-425.[Abstract/Free Full Text]
- Schwerzmann M, Salehian O, Elliot T, Merchant N, Siu SC, Webb GD. Images in cardiovascular medicine. Anomalous origin of the left coronary artery from the main pulmonary artery in adults: coronary collateralization at its best. Circulation 2004;110:e511-e513.[Free Full Text]
- Chan RK, Hare DL, Buxton BF. Anomalous left main coronary artery arising from the pulmonary artery in an adult: treatment by internal mammary artery grafting J Thorac Cardiovasc Surg 1995;109:393-394.[Free Full Text]
- Sabiston Jr DC, Neill CA, Taussig HB. The direction of blood flow in anomalous left coronary artery arising from the pulmonary artery Circulation 1960;22:591-597.[Abstract/Free Full Text]
- Backer CL, Stout MJ, Zales VR, et al. Anomalous origin of the left coronary artery. A twenty-year review of surgical management. J Thorac Cardiovasc Surg 1992;103:1049-1057.[Abstract]
- Collins N, Colman J, Benson L, Hansen M, Merchant N, Horlick E. Successful percutaneous treatment of anomalous left coronary artery from pulmonary artery Int J Cardiol 2007;122:e29-e31.[Medline]
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Abstract
Introduction
