Ann Thorac Surg 2006;81:1127-1129
© 2006 The Society of Thoracic Surgeons
Case report
Surgical Management of Single Ostium Anomalous Coronary Artery From Pulmonary Artery
Qiang Chen, MD
a
,
Eleanor Soo, MRCS
a
,
Andrew J.P. Tometzki, FRCP
b
,
Andrew J. Parry, FRCS
a
,
*
a Department of Pædiatric Cardiac Surgery, Bristol Royal Hospital for Children, Bristol, England
b Department of Pædiatric Cardiology, Bristol Royal Hospital for Children, Bristol, England
Accepted for publication December 28, 2004.
* Address correspondence to Dr Parry, Department of Pædiatric Cardiac Surgery, Bristol Royal Hospital for Children, Upper Maudlin Street, Bristol, BS2 8BJ, England (Email: aj_parry{at}yahoo.co.uk).
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Abstract
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A 6-week-old infant presenting in heart failure was suspected on echocardiography of having anomalous origin of the whole coronary circulation from the pulmonary artery arising from a single ostium. This was confirmed at operation, and the child underwent successful correction. The management of cardiopulmonary bypass and myocardial protection in this abnormality is discussed.
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Introduction
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A totally pulmonary artery (PA) dependent coronary circulation is a rare, life-threatening condition. Although originally reported in 1931, premortem diagnosis was not made until 1976 [1]. Since then, despite many attempts, only two successful surgical corrections have been reported [2]. We report the successful surgical treatment of this anomaly in a child in whom the morphology was demonstrated by preoperative echocardiogram.
A 6-week-old child was admitted with failure to thrive and respiratory distress. An echocardiogram showed a dilated, poorly functioning left ventricle with fractional shortening of 14%. There was mild mitral regurgitation and moderate tricuspid regurgitation at a velocity of 4 m/s. Careful examination of the great vessel roots showed antegrade flow in a "left" coronary artery originating from the PA (Fig 1A), which was surmised to be due to severe pulmonary hypertension; the right coronary could not be clearly identified. Intracardiac anatomy was normal.
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Fig 1. The short-axis echocardiogram clearly demonstrates antegrade flow down the single coronary artery that arises from (A) the left side of the pulmonary root and (B) divides almost immediately (A = anterior; Ao = aorta; L = left; LCA = left coronary artery; P = posterior; PA = pulmonary artery; R = right; RCA = right coronary artery.)
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At operation, on opening the pericardium, it was immediately apparent that there could be no systemically dependent cardiac perfusion, as the whole of the myocardium, particularly the right ventricle, was blue as in complete transposition of the great arteries. Inspection of the anterior aortic root failed to identify a right coronary artery, and the conal arteries were seen to be running directly inferiorly from the left side of the PA. A single coronary artery was found to originate from the left lateral border of the main PA remote from the aorta, with a very short main trunk and early trifurcation (Fig 1B). Marking stitches were placed in the ascending aorta and the main PA marking where they were naturally opposed.
The ascending aorta was cannulated with an 8F cannula, and the PA root with a 6F cannula, while the superior vena cava and inferior vena cava were cannulated in a standard fashion. Bypass was commenced perfusing down both arterial cannulas, and the branch pulmonary arteries were immediately snared to prevent runoff into the pulmonary vascular bed with resultant myocardial ischemia. The left ventricle was vented. The heart was arrested using cold crystaloid cardioplegia delivered directly into both the aortic root (in case there were some small branches) and down the PA root cannula. An aortopulmonary window was created between the aorta and the PA centered on the marking stitch, and a patch of pericardium was used to create the tunnel between the window and coronary ostium as described previously [3]. The PA was reconstructed with a pericardial patch. Cardiopulmonary bypass was weaned on dopamine and low-dose adrenaline. The postoperative course was uneventful, and the infant was extubated 4 days postoperatvely. He was discharged on the 14th postoperative day, at which time fractional shortening had improved to 22%.
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Comment
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Single ostium anomalous coronary artery from the PA is rapidly lethal, and patients often present in early infancy with infarction and sudden death [4]. Associated cardiac defects occur in as many as 68% of cases [4], and these may permit longer survival. Even when the diagnosis is made premortem, the outlook remains poor; despite several reported attempts at correction, in only 2 cases was intervention successful [2].
An accurate appreciation of the anatomy is crucial in the successful management of these children. In our patient, preoperatively, two factors suggested the possibility of a totally PA dependent coronary circulation. First, the child was aged only 6 weeks. This young age of presentation is a consistent feature throughout the literature, with 84% of patients presenting within 3 days of life and 60% dying within 2 weeks [4]. This compares with the later age of presentation, as is seen with anomalous left coronary artery from the pulmonary artery (ALCAPA). Second, antegrade flow was observed on echocardiography in the anomalous coronary artery, although this was initially considered to be due to severe pulmonary hypertension. Previously, making the diagnosis has required cardiac catheterization, which is not routine in this clinical scenario. Intraoperatively, the diagnosis was suggested by the color of the myocardium. The diagnosis was confirmed by exploring the aortic root.
The management of bypass in this setting is critical for successful outcome. The instigation of bypass in a standard manner with no means of supporting the coronary circulation will inevitably lead to a period of ischemia in an already severely compromised ventricle. We therefore advocate dual cannulation and perfusion of both aorta and PA root, as this will stabilize myocardial perfusion, and the administration of cardioplegia into both aortic and PA roots to ensure optimal myocardial protection. Indeed, we advocate this approach in all cases of ALCAPA.
The ideal method of achieving aortic origin of the coronary blood flow is by direct coronary transfer, where possible, as baffles within the pulmonary artery may potentially lead to pulmonary stenosis. In our case, however, concerns about kinking the single arterial trunk caused us to perform a successful repair using an intrapulmonary artery tunnel. However, the primary goal must be to achieve unobstructed coronary flow by whichever means are necessary.
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References
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- D'Alessandro LC, Di Lorenzo M. Coronaria unica con origine dall'arteria polmonare sinistra in un uomo di 39 anni ancora vivente, affetto da ‘truncus arteriosus communis' G Ital Cardiol 1976;6:939-945.[Medline]
- Urcelay GE, Iannettoni MD, Ludomirsky A, et al. Origin of both coronary arteries from the pulmonary artery Circulation 1994;90:2379-2384.[Abstract/Free Full Text]
- Hamilton DI, Ghosh PK, Donnelly RJ. An operation for anomalous origin of left coronary artery Br Heart J 1979;41:121-124.[Abstract/Free Full Text]
- Heifetz SA, Robinowitz M, Mueller KH, Virmani R. Total anomalous origin of the coronary arteries from the pulmonary artery Pediatr Cardiol 1986;7:11-18.[Medline]
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Abstract
Introduction
