A rare case of anomalous origin of the left main coronary artery in an adult patient
© Dionne et al.; licensee BioMed Central Ltd. 2013
Received: 18 August 2012
Accepted: 11 January 2013
Published: 22 January 2013
Anomalous origin of left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly that causes a left-to-right shunt via the coronary system, resulting in coronary steal. We report an unusual case of a healthy 48 years-old patient presenting with dyspnea on exertion and mild chest pain who underwent surgical correction of this rare anomaly. Multiple procedures have been proposed in adults with ALCAPA. Although re-implantation of the left main coronary artery (LMCA) to the aorta remains the most physiological correction for this anomaly, the combination of LMCA ligation and coronary artery bypass grafting provides a dual coronary flow system and is preferable when re-implantation is impossible.
KeywordsCongenital heart defect Cardiac surgery Coronary artery bypass graft
Anomalous origin of left coronary artery from the pulmonary artery (ALCAPA) is an uncommon congenital anomaly affecting 1/300,000 live births . As pulmonary artery (PA) pressure lowers to a third that of systemic pressure during the neonatal period, left coronary flow will tend to reverse into the pulmonary artery (PA) resulting in coronary steal and a left to right shunt, with subsequent decreased myocardial perfusion and volume overload. The vast majority of patients present with heart failure secondary to myocardial ischemia or die during infancy. Because patients with symptoms should be operated, it is seldom identified in adults. Survivors have developed significant collateral flow from the right coronary artery (RCA) and secondary dilatation of the coronary arteries.
We present herein an unusual case of ALCAPA seen in one of the oldest patient ever reported.
Additional file 1: Video 1.mov. Intra-operative video showing the increase in size of the tortuous coronaries on the beating heart. (MOV 4 MB)
This exceptional case of a late presentation of ALCAPA was not only interesting from a clinical and imaging perspective but also from a therapeutic standpoint. Since patients with this anomaly rarely survive past infancy without surgical correction, very few adult patients have been reported in the literature, especially after 40 years old. We believe that patients can survive through adulthood with this anomaly without significant myocardial damage because of the development of an important collateral flow from the RCA.
Multiple procedures have been proposed in adults with ALCAPA including ligation of the left main coronary artery (LMCA), re-implantation of the LMCA to the aorta, creation of a baffle through the pulmonary artery (Takeuchi procedure) and a combination of LMCA ligation and CABG. Although re-implantation of the LMCA to the aorta remains the most physiological correction for this anomaly, the combination of LMCA ligation and CABG provides a dual coronary flow system and is preferable when re-implantation is impossible [2, 3].
In this case, re-implantation of the LMCA into the aorta was considered unfeasible because of the distance between the insertion site of the LMCA on the PA and the aorta. The Takeuchi procedure was also considered but discarded because of the reported increased risk of supra-valvular stenosis as the distance increases between the insertion site of the LMCA and the junction between the aorta and the pulmonary artery . End-to-end anastomosis of the LMCA with the aorta using an interposition arterial graft was not achieved because of the frailty of the surrounding tissues, the numerous collateral branches, and the inability to mobilize the bypassed vessel sufficiently to achieve an hemodynamically favorable end-to-end anastomosis. Creation of a tube graft using pulmonary artery wall autograft, as described by Wu et al. , with a remote LMCA insertion site in regards to the aorta, could have been another valuable option.
The collateral flow in this patient was impressive. Following LMCA ligation on CPB, cardiac arrest was not attained despite adequate cool antegrade and retrograde cardioplegia flow and relative hypothermia. In order to match the size of the large LAD, allow unrestricted flow and avoid competitive flow between the graft and the collaterals from the RCA, the largest possible conduit was selected. The choice of a large SVG conduit over an arterial graft (such as the internal thoracic or the radial artery) was in response to this situation.
In a series of 6 adult patients who underwent saphenous vein bypass grafting and direct ALCAPA closure from inside the PA, Moodie and associates reported a graft patency rate of 80% at a mean follow-up of 5.8 years . Furthermore, 10-year patency of a SVG was found to be 88% when grafted to a large size LAD (larger than 2.0 mm), as reported by Goldman et al. . Moreover, we obtained a SVG flow of 117 ml/min as measured at the time of surgery, which is significantly higher than what we routinely measure in LITA pedicles grafted to the LAD. We believe that this high blood flow in the SVG, along with dual antiplatelet treatment (AAS and Clopidogrel) will result in excellent long-term patency.
Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Anomalous origin of left coronary artery from the pulmonary artery
Coronary artery bypass graft
Cardiac computed tomography angiography
Left main coronary artery
Right coronary artery.
- Erdinc M, Hosgor K, Karahan O: Repair of anomalous origin of the left coronary artery arising from right pulmonary artery with rolled-conduit-extended reimplantation in an adult. J Card Surg. 2011, 26: 604-607. 10.1111/j.1540-8191.2011.01337.x.View ArticlePubMedGoogle Scholar
- Dodge-Khatami A, Mavroudis C, Backer CL: Anomalous origin of the left coronary artery from the pulmonary artery: Collective review of surgical therapy. Ann Thorac Surg. 2002, 74: 946-955. 10.1016/S0003-4975(02)03633-0.View ArticlePubMedGoogle Scholar
- Bunton R, Jonas RA, Lang P, Rein AJ, Castaneda AR: Anomalous origin of left coronary artery from pulmonary artery. Ligation versus establishment of a two coronary artery system. J Thorac Cardiovasc Surg. 1987, 93: 103-108.PubMedGoogle Scholar
- Ginde S, Earing MG, Bartz PJ, Cava JR, Tweddell JS: Late Complications After Takeuchi Repair of Anomalous Left Coronary Artery From the Pulmonary Artery: Case Series and Review of Literature. Pediatr Cardiol. 2012, 33 (7): 1115-1123. 10.1007/s00246-012-0260-5.View ArticlePubMedGoogle Scholar
- Wu QY, Xu ZH: Surgical treatment of anomalous origin of coronary artery from the pulmonary artery. Chin Med J. 2008, 121 (8): 721-724.PubMedGoogle Scholar
- Moodie DS, Fyfe D, Gill CC: Anomalous origin of the left coronary artery from the pulmonary artery (Bland- White-Garland syndrome) in adult patients: long-term follow- up after surgery. Am Heart J. 1983, 106: 381-388. 10.1016/0002-8703(83)90207-7.View ArticlePubMedGoogle Scholar
- Goldman S, Zadina K, Moritz T, Ovitt T, Sethi G, Copeland JG, Thottapurathu L, Krasnicka B, Ellis N, Anderson RJ, Henderson W: Long-Term Patency of Saphenous Vein and Left Internal Mammary Artery Grafts After Coronary Artery Bypass Surgery. JACC. 2004, 44 (11): 2149-2156. 10.1016/j.jacc.2004.08.064.View ArticlePubMedGoogle Scholar
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