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      Anomalous origin of the left circumflex coronary artery from the pulmonary artery. A very rare congenital anomaly in an adult patient diagnosed by cardiovascular magnetic resonance

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          Abstract

          Here we report for the first time on the diagnostic potential of cardiovascular magnetic resonance (CMR) to delineate the proximal course of an anomalous left circumflex coronary artery (LCX) originating from the right pulmonary artery in an adult patient with no other form of congenital heart disease. The patient was referred to our institution due to exertional chest discomfort. X-Ray coronary angiography showed a normal left anterior descending coronary artery (LAD) and right coronary artery (RCA), while the LCX was filled retrograde by collateral flow through the LAD and the RCA. The origin of the LCX was postulated to be the pulmonary artery, but the exact origin of the anomalous artery could not be depicted on conventional angiograms. CMR provided the unambiguous depiction of the origin of the anomalous LCX from the right pulmonary artery and the delineation of its proximal course in this case of a very rare coronary anomaly in adults.

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          Magnetic resonance angiography of anomalous coronary arteries. A new gold standard for delineating the proximal course?

          The clinical significance of anomalously originating coronary arteries depends on their proximal course. Diagnosis of this course by conventional x-ray coronary angiography alone may be equivocal. We postulated that with fast magnetic resonance (MR) angiography, accurate detection of anomalous coronary arteries and unambiguous delineation of their proximal course is feasible. In a selected group of 38 patients, 19 of them having an anomalously originating coronary artery, a fast MR angiographic technique was used to study the proximal coronary anatomy. Blinded analysis of randomly ordered MR studies was performed independently by two observers. Both origin and proximal course of the coronary arteries were defined. Two cardiologists reviewed all x-ray coronary angiograms. After the separate analyses, a final consensus result was defined for each patient. In 37 patients, successful MR coronary angiography could be performed. Interobserver agreement for determining both origin and proximal course was 100%. An x-ray coronary angiogram was available in 36 patients. In 3 patients (all with an anomalous left main coronary artery originating from the right aortic sinus), there was disagreement about the proximal course between the results of MR and x-ray coronary angiography. Review of these cases demonstrated that MR angiography had unambiguously visualized the proximal coronary artery course, whereas the results of x-ray angiography had been equivocal. Thus, sensitivity and specificity for detecting anomalous coronary arteries and delineating their proximal course were 100%. These data suggest that fast MR angiography is highly accurate in determining the origin and delineating the proximal course of anomalous coronary arteries, even in those cases in which x-ray coronary angiographic diagnosis is difficult or even erroneous.
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            Anomalous left circumflex coronary artery from the right pulmonary artery: first adult case report.

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              Associated coronary and cardiac anomalies in the tetralogy of Fallot. An angiographic study.

              Numerous studies have pointed out the frequent association of tetralogy of Fallot (TF) with other cardiovascular defects and coronary tree anomalies. We found cardiac defects in 181 (68%) out of 265 patients with TF investigated by catheterization and selective coronary angiography. These anomalies were isolated in 88 cases (49%) and associated with others in 93 patients. In the case of an isolated anomaly associated with TF, the coronary tree was involved in 37.5% and the cardiovascular system in the remaining 62.5%; in the case of two anomalies, the coronary system was involved in 66% of the patients and the cardiovascular apparatus in 34%; in the case of three or more anomalies, the coronary arteries were involved in 71% and the cardiovascular system in 29%. Anomalies in the course and/or distribution of coronary arteries were present in 96 patients (36%): 10 had a single coronary ostium, 13 a left anterior descending artery arising from the right coronary artery, one a circumflex artery arising from the right coronary artery. Small fistulas between coronary arteries and the pulmonary artery were found in 20 cases; anastomoses between coronary and bronchial arteries or right atrium in 42. In 39 patients we observed a large conus artery or large anterior ventricular branches crossing the right ventricle. A right aortic arch was found in 56 patients (21%), a stenosis of the trunk and/or the peripheral pulmonary artery in 35 (13%) and pulmonary artery atresia in five. Four patients showed a complete atrioventricular canal, three an atrial septal defect (primum type) with cleft of the mitral valve, 61 (23%) an atrial septal defect (ostium secundum). Eleven patients had anomalies of the systemic venous return, 26 (10%) a patent ductus arteriosus. Four patients had valvular abnormalities. In our series, a large proportion of cardiac defects associated with TF consists of anomalies of coronary arteries. Our data confirm the usefulness of performing preoperatively routine coronary angiography in patients with complex congenital heart disease.
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                Author and article information

                Journal
                J Cardiovasc Magn Reson
                Journal of Cardiovascular Magnetic Resonance
                BioMed Central
                1097-6647
                1532-429X
                2008
                21 January 2008
                : 10
                : 1
                : 4
                Affiliations
                [1 ]Department of Cardiology, University of Heidelberg, Heidelberg, Germany
                Article
                1532-429X-10-4
                10.1186/1532-429X-10-4
                2244609
                18272006
                9bcfeb98-cd0f-431f-8329-e8c9cfd75cde
                Copyright © 2008 Korosoglou et al; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 13 December 2007
                : 21 January 2008
                Categories
                Case Report

                Cardiovascular Medicine
                Cardiovascular Medicine

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