Ann Thorac Surg 1998;65:248
© 1998 The Society of Thoracic Surgeons
Case Reports
Should We Address the Course as Well as the Origin of a Translocated Anomalous Coronary Artery?
Michael D. Black, MD,
Brian W. McCrindle, MD,
Robert M. Freedom, MD
Division of Cardiovascular Surgery, The Hospital for Sick Children and The University of Toronto, Toronto, Ontario, Canada
Division of Cardiology, The Hospital for Sick Children and The University of Toronto, Toronto, Ontario, Canada
Accepted for publication August 29, 1997.
Dr Black, Division of Cardiovascular Surgery, The Hospital for Sick Children, 555 University Ave, Toronto, ON, Canada M5G 1X8 (e-mail: michael.black@mailhub.sickkids.on.ca).
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Abstract
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The identification of an anomalous left coronary artery arising from the pulmonary artery demands urgent surgical attention. Myocardial infarction and ongoing myocardial ischemia are a direct consequence with subsequent left ventricular dysfunction. A modification using a combination of autogenous aortic and pulmonary artery flaps is described, which addresses both the origin and the course of the anomalous coronary artery—until now, a feature not generally considered necessary of repairs involving anomalous left coronary artery arising from the left facing pulmonary sinus.
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Introduction
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Although rare, anomalous origin of the left coronary artery from the pulmonary artery (ALCA), in time, is lethal. Most infants and children succumb to severe left ventricular dysfunction or dysrhythmias. Sudden death is common, and subsequent autopsy may reveal this unsuspected congenital anomaly. Urgent surgical intervention is thus recommended. However, direct anastomosis of the anomalous artery to the aorta is not always feasible. Because the location of the ALCA varies in origin from the pulmonary artery, so should the repair strategy. Turley and associates [1] suggest aortic implantation is possible in all cases of ALCA from the pulmonary artery with autogenous tunnels constructed from the pulmonary artery. However, the course of the translocated ALCA may still be abnormal, lying anterior to the pulmonary artery, and thus vulnerable to compression during periods of increased cardiac output.
We would like to further refine the transfer technique of the ALCA originating from the left facing pulmonary valve sinus by suggesting an approach that directs the left main coronary artery in a retropulmonary position. Autologous tissue tunnels should negate future conduit changes and avoid intraarterial baffles. Avoidance of late right ventricular outflow tract obstruction by external conduit "bow-string" compression or intraarterial baffle obstruction should thus be possible. Moreover, autogenous tunnels previously described require a circumferential anastomosis of ALCA to the aorta upon translocation; thus late ostial stenosis remains a theoretical hazard. This double-flap technique could avoid the latter complication because at least half of the neo-coronary ostium is composed of aorta.
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Surgical Technique
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The infant is prepared and draped in a supine position. The heart is exposed via a median sternotomy, a longitudinal pericardiotomy, and a subtotal thymectomy. Cardiopulmonary bypass is established by cannulating the ascending aorta and the venae cavae. Cooling is initiated to a nadir temperature of approximately 25°C. A left ventricular vent is placed via the right superior pulmonary vein to prevent left ventricular distention. The pulmonary arteries are snared to maintain elevated pulmonary vascular resistance, thus reducing the extent of pulmonary runoff and subsequent coronary steal. The coronary artery anatomy is verified, ie, left main coronary artery originating from the left facing pulmonary valve sinus. A large gap between the ALCA and the aorta should be anticipated—too far to reach by primary translocation and reimplantation.
The aorta and pulmonary arteries are cross-clamped and cardioplegia administered antegrade via both great vessels, thereby providing optimal myocardial protection. Once the heart is arrested in diastole, the main pulmonary artery is transected proximal to the confluence of the branch pulmonary arteries. Thereafter, a large button of pulmonary arterial tissue encompassing the left main coronary ostia is removed (representing the anterior portion of the proposed autologous tunnel). The left pulmonary sinus is ultimately replaced with autologous pericardium treated in glutaraldehyde. A"trap-door" type incision is created in the anterior portion of the aorta just distal to the valve commissures (representing the posterior flap of the autologous tunnel) (Fig 1). Adequate visualization of the aortic valve and the right coronary artery ostia is essential to avoid iatrogenic damage. The tunnel is sutured with an absorbable monofilament 7-0 suture. A piece of autologous pericardium treated in glutaraldehyde repairs the remaining defect in the anterolateral aorta (Fig 2). Both left coronary arteries are inspected to ascertain probe-patency, and once the left side of the heart is deaired, the cross-clamp is removed. Of note in our case, the anterolateral wall of the left ventricle had extensive fibrosis from previously unrecognized myocardial infarctions.
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Repair of anomalous origin of the left main coronary artery from the left-facing pulmonary sinus (A). A large, posterior-based "trap-door" incision is created in the aorta (AO), just above the sinotubular ridge, representing the posterior wall of neo-coronary tunnel. The entire left-facing pulmonary sinus is removed with a portion of the adjacent anterior pulmonary artery (PA), representing the anterior wall of the neo-coronary tunnel (B).
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The tunnel is completed with both autologous flaps (A). The left-facing pulmonary sinus is replaced with autologous pericardium. The pulmonary artery (PA) is reconstituted in an end-to-end fashion (B). (AO = aorta.)
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The pulmonary artery is now closed primarily without tension by ligation and division of the ligamentum/ductus arteriosus and by mobilization of the branch pulmonary arteries to their respective pulmonary hila. Our patient was successfully weaned from cardiopulmonary bypass and discharged home on her seventh postoperative day. Echocardiographic examination demonstrated a widely patent left coronary artery with preserved and slightly improved myocardial function. Delayed improvement in left ventricular function should be anticipated.
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Comment
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Few would argue that surgical repair of ALCA arising from the pulmonary artery should involve creation of a two-vessel coronary system [2][3][4]. Because this is a disease of childhood, autogenous tunnels would seem most appropriate, thus avoiding synthetic conduits, intrapulmonary tunnels, or bypass grafts. Left main coronary tunnels should thus maintain their size proportional to somatic growth. Although the origin of the anomalous left coronary artery has been previously addressed [5][6], correcting the course of the anomalous artery generally has not been considered necessary [7]. Of significance, sudden death is not uncommon among young adults with an abnormal course of their coronary arteries (usually found between the great arteries). Dilatation of the pulmonary artery during high output states, such as exercise, may recreate in essence the lethal situation described above by directing the left main coronary artery anterior to the pulmonary artery. This potentially lethal situation holds especially true when the coronary artery to be translocated arises from the left-facing pulmonary valve sinus and thus is limited to translocation into the proximal anterior aorta [5][8].
The described technique of coronary transfer addresses both origin and course of ALCA arising from the pulmonary artery (left-facing sinus). In addition, left main ostial stenosis should be minimized as at least half of the ostia’s circumference is composed of aorta. Our patient did exceedingly well, not requiring either delayed chest closure or ventricular assist. However, a significant dated anterolateral infarct was identified. Hopefully with anticipated somatic growth this damaged area should become less hemodynamically significant. Long-term follow-up will be required to ascertain patency and growth of the newly implanted coronary artery using this double-flap technique.
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Addendum
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The technique described above has again been successfully employed in a 4-year-old child presenting with signs and symptoms of myocardial dysfunction secondary to ALCA.
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Acknowledgments
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We acknowledge Mr Phil Dakin for his artistic contributions.
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References
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- Turley K, Szarnicki RJ, Flachsbart KD, Richter RC, Popper RW, Tarnoff H Aortic implantation is possible in all cases of anomalous origin of the left coronary artery from the pulmonary artery. Ann Thorac Surg 1995;60:84-89.[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;103:1049-1058.[Abstract]
- Vouhé PR, Baillot-Vernant F, Trinquet F, et al. Anomalous left coronary artery from the pulmonary artery in infants: which operation? When?. J Thorac Cardiovasc Surg 1987;94:192-199.[Abstract]
- Alexi-Meshishvili V, Hetzer R, Weng Y Anomalous origin of the left coronary artery from the pulmonary artery. Early results with direct aortic reimplantation. J Thorac Cardiovasc Surg 1994;108:354-362.[Abstract/Free Full Text]
- Sese A, Imoto Y New technique in the transfer of an anomalously originated left coronary artery to the aorta. Ann Thorac Surg 1992;53:527-529.[Abstract/Free Full Text]
- Dua R, Smith JA, Wilkinson JL, et al. Long-term follow-up after two coronary repair of anomalous left coronary artery from the pulmonary artery. J Card Surg 1993;8:384-390.[Medline]
- Kirklin JW, Barratt-Boyes BG Anomalous connection of the left coronary artery to the pulmonary artery. In: Kirklin JW, Barratt-Boyes BG, eds. Cardiac surgery. New York: Churchill Livingstone, 1993:1177-1188.
- Roberts WC Adult congenital heart disease. Philadelphia: F.A. Davis, 1987:583-629.
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Abstract
Introduction
