Ann Thorac Surg 2006;82:2285-2287
© 2006 The Society of Thoracic Surgeons
Case Reports
Anomalous Left Main Coronary Artery From the Main Pulmonary Artery in an Elderly Patient
Amit Korach, MDa,
Praveen Menon, MDa,
Mandeep Dhadly, MDb,
Khether E. Raby, MDb,
Richard J. Shemin, MDa,
Oz M. Shapira, MDa,*
a Department of Cardiothoracic Surgery, Boston Medical Center, Boston, Massachusetts
b Evans Department of Medicine, Section of Cardiology, Boston Medical Center, Boston, Massachusetts
Accepted for publication April 14, 2006.
* Address correspondence to Dr Shapira, Department of Cardiothoracic Surgery, Suite B-402, Boston Medical Center, 88 E. Newton St, Boston, MA 02118. (Email: oz.shapira{at}bmc.org).
Presented at the Breakfast Session of the Forty-second Annual Meeting of The Society of Thoracic Surgeons, Chicago, IL, Jan 30–Feb 1, 2006.
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Abstract
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Anomalous origin of the left main coronary from the pulmonary trunk in an elderly patient is extremely rare. We report a 73-year-old woman who presented with new onset of angina and atrial fibrillation. Evaluation revealed anomalous origin of the left main coronary artery from the main pulmonary artery and tight proximal left anterior descending coronary artery stenosis. The patient underwent primary closure of the anomalous left main coronary artery orifice within the pulmonary artery, aorta-to-left anterior descending coronary artery saphenous vein bypass grafting and Maze procedure. Six months postoperatively the patient was asymptomatic and a Thallium stress test was negative for ischemia.
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Introduction
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Anomalous origin of the left main coronary artery from the main pulmonary artery is a rare congenital anomaly that usually presents in the neonatal or pediatric age group with symptoms of ischemia or congestive heart failure, and is associated with 90% mortality if not surgically corrected [1]. Presentation in the adult is extremely rare [2]. We describe an elderly patient who presented with this anomaly and discuss the diagnosis and various surgical approaches to this rare entity.
A 73-year-old woman presented with chest discomfort and dyspnea during mild physical activity. Her past medical history was remarkable for remote myocardial infarction, noninsulin-dependent diabetes mellitus, hypertension, dyslipidemia, and permanent atrial fibrillation.
A Thallium stress test was positive for angina, ST-T segment changes, and reversible anterior wall perfusion defect. Echocardiogram revealed a left ventricle ejection fraction of 40% with anterior wall hypokinesis. Right heart catheterization demonstrated O2 saturation step-up from 49% in the right ventricle to 61% in the pulmonary artery with a Qp/Qs shunt ratio of 1.3:1. Coronary angiography (Fig 1) and rapid sequence computed tomographic angiography (Fig 2) showed a left main coronary artery (LMCA) that originated from the main pulmonary artery (MPA). The lifelong arterial-venous fistula resulted in gigantic, tortuous coronary arteries. A markedly dilated (1.0 cm) left anterior descending coronary artery (LAD) had a tight proximal stenosis and was filled by collaterals from a dilated (2.0 cm) tortuous right coronary artery. The patient was operated on through a median sternotomy. An "off-pump" high-intensity-focused-ultrasound Maze procedure was performed. Repair was then performed using cardiopulmonary bypass and cardioplegic arrest. A longitudinal arteriotomy was performed in the proximal MPA, and a large (0.9 cm) anomalous orifice of the LMCA was identified just above the pulmonary valve. The anomalous orifice was closed primarily using a continuous polypropylene suture. The MPA was closed and an aorta-to-distal LAD reversed saphenous vein graft was constructed. The patient was weaned off cardiopulmonary bypass without difficulty.
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Fig 1. Coronary angiography demonstrating very dilatated, tortuous coronary arteries. In the right panel a tight proximal stenosis (arrow) is observed in the left anterior descending coronary artery (LAD) that is filled by collaterals from the right coronary artery (RCA, left panel), and is drained into the main pulmonary artery (Main PA, right panel). (PDA = posterior descending coronary artery.)
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Fig 2. Computed tomographic scan demonstrating anomalous origin of the left main coronary artery (arrow) from the main pulmonary artery (MPA). (Ao = aorta.)
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The postoperative course was uneventful. Six months after the operation the patient was in New York Heart Association functional class I, and a stress test was negative for myocardial ischemia.
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Comment
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Anomalous origin of the left main coronary artery from the pulmonary artery, also known as Bland-White-Garland syndrome, is a rare congenital cardiac anomaly affecting approximately 1 in 300,000 live births. The mortality rate in the pediatric population exceeds 90% without surgical intervention [1].
Symptoms usually occur in the first few months of life. Late presentation in the adult or elderly is extremely rare [2]. We believe that this patient is the oldest reported in the literature. Early on the patient had a large network of collaterals develop that prevented myocardial ischemia. In fact, during her life from birth forward to the operation the entire heart was supplied by a single vessel (ie, a gigantic [2.0 cm in diameter] right coronary artery). However, later in life with the development of coronary artery disease and tight proximal LAD stenosis, her symptoms appeared. Most likely proximal LAD stenosis, although decreasing the shunt, resulted in an "outflow" obstruction to coronary flow with decreased perfusion and myocardial ischemia.
Surgery is considered the treatment of choice for this anomaly. Several approaches have been described. Isolated ligation of the LMCA is the simplest approach. It decreases the left-to-right shunt, but is associated with persistent left ventricle dysfunction and higher incidence of sudden death [3]. More attractive approaches are those resulting in double coronary artery systems. These include ligation of the LMCA and coronary artery bypass grafting, baffling of the LMCA within the MPA, and detachment of the LMCA from the MPA with reimplantation to the proximal aorta [4, 5]. These techniques eliminate the left-to-right shunt and improve myocardial perfusion with better preservation of left ventricular function. The optimal surgical approach varies based on individual patient anatomy. Reimplantation of the LMCA into the aorta has been the most frequently used technique in the pediatric age group, and ligation of the anomalous artery with coronary artery bypass grafting has been the preferred approach in the adult [3–6].
Given the gigantic, thin wall coronary arteries with tight proximal LAD stenosis we elected to primarily close the orifice of the LMCA within the MPA and graft the LAD. This deemed to be the simplest approach to eliminate the shunt and establish a double coronary system. We preferred the saphenous vein rather than the left internal mammary artery to graft to the LAD to avoid hypoperfusion syndrome, being concerned that a relatively small-caliber left internal mammary artery will not provide adequate flow to this very large (1.0 cm) LAD in the immediate postoperative period. The circumflex coronary artery was not grafted, as it was very small and well collateralized.
In summary, the association coronary artery disease with an anomalous left main coronary artery from the main pulmonary artery is a rare cause of myocardial ischemia in the elderly. Primary closure of the orifice of this anomalous artery within the main pulmonary artery and coronary artery bypass grafting is a simple, safe, and effective approach to this rare entity.
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References
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- Wesselhoeft H, Fawcett JS, Johnson AL. Anomalous origin of the left coronary artery from the pulmonary trunkIts 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]
- Khanna A, Torigian DA, Ferrari VA, Bross RJ, Rosen MA. Anomalous origin of the left coronary artery from the pulmonary artery in adulthood on CT and MRI AJR 2005;185:326-329.[Free Full Text]
- Backer CL, Stout MJ, Zales VR, et al. Anomalous origin of the left coronary arteryA twenty-year review of surgical management. J Thorac Cardiovasc Surg 1992;103:1049-1057.[Abstract]
- Takeuchi S, Imamura H, Katsumoto K, et al. New surgical method for repair of anomalous left coronary artery from pulmonary artery J Thorac Cardiovasc Surg 1979;78:7-11.[Abstract]
- Schwartz ML, Jonas RA, Colan SD. Anomalous origin of left coronary artery from pulmonary artery: recovery of left ventricular function after dual coronary repair J Am Coll Cardiol 1997;30:547-553.[Abstract]
- Ando M, Mee RB, Duncan BW, Drummond-Webb JJ, Seshadri SG, Igor Mesia CI. Creation of a dual-coronary system for anomalous origin of the left coronary artery from the pulmonary artery utilizing the trapdoor flap method Eur J Cardiothorac Surg 2002;22:576-581.[Abstract/Free Full Text]
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Abstract
Introduction
