Ann Thorac Surg 1999;67:233-235
© 1999 The Society of Thoracic Surgeons
Case Reports
Aortopulmonary window with anomalous origin of the right coronary artery
Jürg Grünenfelder, MDa,
Gregor Zünd, MDa,
Paul R. Vogt, MDa,
Marko I. Turina, MDa
a Clinic of Cardiovascular Surgery, University Hospital, Zürich, Switzerland
Accepted for publication June 11, 1998.
Address reprint requests to Dr Grünenfelder, Clinic of Cardiovascular Surgery, University Hospital, Rämistrasse 100, 8091 Zürich, Switzerland
e-mail: (juerg.gruenenfelder{at}chi.usz.ch)
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Abstract
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We report a case of an aortopulmonary window with a right coronary artery arising from the pulmonary trunk. This exceedingly rare anomaly with anomalous coronary artery presented without myocardial ischemia owing to the aortopulmonary window. The correct diagnosis was made by angiography and a successful surgical correction was performed.
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Introduction
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Aortopulmonary window is a rare but well-described congenital heart disease. However, the combination of aortopulmonary window with an anomalous origin of the right coronary artery from the pulmonary trunk is extremely rare and only about 8 cases with the same anomaly have been published worldwide [1–8]. The purpose of this paper is to present a literature review (Table 1) and a new case of this rare abnormality with the physiologic effects and its surgical correction.
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Table 1. Literature Review of Cases with Aortopulmonary Window and Anomalous Origin of Coronary Arteries From Pulmonary Artery
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A 3-week-old male infant was admitted with history of intermittent tachypnea and failure to thrive. On angiography there was a 4-mm aortopulmonary window between the inner curvature of the ascending aorta and the pulmonary trunk (Fig 1). The mean arterial pressure in the aorta was 45 mm Hg and 31 mm Hg in the main pulmonary artery with an O2 saturation of 100% and 96%, respectively. A patent ductus arteriosus was present as well as a small atrial septal defect. The left coronary artery was normal, but the right coronary artery (RCA) originated anomalously from the right anterior aspect of the main pulmonary artery proximal to the window (Fig 2).
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Fig 1. Left ventricular injection in left anterior oblique projection showing a 4-mm aortopulmonary window in the inner curvature of the ascending aorta.
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Fig 2. Injection of the main pulmonary artery revealed anomalous origin of right coronary artery from the anterior aspect of the pulmonary trunk.
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The operation was performed on cardiopulmonary bypass with deep hypothermia and circulatory arrest. The border of the window and the ostium of the RCA were divided, and the defect in the pulmonary trunk was closed with a pericardial patch. The aorta was repaired by direct suture. The ostium of the RCA was excised as a button and reanastomosed to the anterior wall of the ascending aorta. The patent ductus arteriosus was ligated using clips. The postoperative course was uneventful.
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Comment
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Aortopulmonary window in association with anomalous origin of the RCA from the main pulmonary artery is extremely rare. From a physiologic standpoint it is also quite different from that seen in patients in whom the RCA anomaly is the sole lesion and therefore myocardial ischemia or infarction is typical. In our patient there was no myocardial ischemia because of the presence of well-oxygenated blood in the pulmonary trunk owing to the left-to-right shunt through the aortopulmonary window as well as the elevated pulmonary artery pressure maintaining adequate perfusion in the anomalous coronary artery. The clinical picture of such patients is dominated by the signs of the left-to-right shunt leading to congestive heart failure and, as a consequence of sustained pulmonary hypertension, to irreversible pulmonary vascular disease.
The principle approach for the operation consists of transferring the RCA from the pulmonary artery to the aorta and closing the window by direct suture or a patch. The procedure described by Luisi and associates [1], in which a flap of the pulmonary wall including the ostium of the RCA was used to close the defect in the aorta, is only feasible if the window lies in close proximity to the anomalous coronary artery. Otherwise stretching of the flap may lead to impaired blood flow through the coronary artery. Because of the slightly anterior location of the coronary artery away from the window we divided the RCA from the pulmonary trunk with a button and translocated the vessel to the adjacent aortic wall. The window in the pulmonary artery was repaired with a pericardial patch whereas the defect at the aortic site could easily be closed with sutures. Special attention was taken to occlude both pulmonary arteries before instituting cardiopulmonary bypass to perfuse both coronaries and to prevent a steal phenomenon from the coronary circulation toward the lungs.
Accurate preoperative diagnosis with either echocardiography or angiography and full understanding of the pathophysiology is key for an optimal surgical correction, which should be achieved early to prevent development of pulmonary vascular obstructive disease.
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References
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Luisi S.V., Ashraf M.H., Gula G., Radley-Smith R., Yacoub M. Anomalous origin of the right coronary artery with aortopulmonary window: functional and surgical consideration. Thorax 1980;35:446-448.[Abstract/Free Full Text]
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Brouwer M.H.J., Beaufort-Krol G.C.M., Talsma M.D. Aortopulmonary window associated with an anomalous origin of the right coronary artery. Int J Cardiol 1990;28:384-386.[Medline]
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Chopra P.S., Reed W.H., Wilson A.D., Rao P.S. Delayed presentation of anomalous circumflex coronary artery arising from pulmonary artery following repair of aortopulmonary window in infancy. Chest 1994;106:1920-1922.[Abstract/Free Full Text]
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Lloyd T.R., Marvin W.J., Lee J. Total anomalous origin of the coronary arteries from the pulmonary artery in an infant with aorticopulmonary septal defect. Pediatr Cardiol 1987;8:153-154.[Medline]
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Vassallo Agius P., Rushworth A., Connolly N. Anomalous origin of left coronary artery from pulmonary artery associated with an aorto-pulmonary septal defect. Br Heart J 1970;32:708-710.[Abstract/Free Full Text]
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Deverall P.B., Lincoln J.C.R., Aberdeen E., Bonham-Carter R.E., Waterston D.J. Aortopulmonary window. J Thorac Cardiovasc Surg 1969;57:479-486.[Medline]
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Faulkner S.L., Oldham R.R., Atwood G.F., Graham T.P. Aortopulmonary window, ventricular septal defect, and membranous pulmonary atresia with a diagnosis of truncus arteriosus. Chest 1974;65:351-353.[Abstract/Free Full Text]
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Doty D.B., Richardson J.V., Falkovsky G.E., Gordonova M.I., Burakovsky V.I. Aortopulmonary septal defect: hemodynamics, angiography, and operation. Ann Thorac Surg 1981;32:244-250.[Abstract]
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Abstract
Introduction
