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      Anomalous Origin of the Entire Coronary System with Separate Ostia within the Right Sinus of Valsalva: A Rare Congenital Anomaly and a Review of the Literature

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          Congenital coronary artery malformations occur infrequently in the general population. In this report, we describe a rare case of anomalous origin of all three coronary arteries from separate ostia within the right coronary sinus.

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          Most cited references 10

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          Anomalous coronary arteries: location, degree of atherosclerosis and effect on survival--a report from the Coronary Artery Surgery Study.

          Limited information is available about clinical presentation, degree of atherosclerosis and effect on overall survival in a large series of patients with coronary artery anomalies. From the National Heart, Lung, and Blood Institute Multicenter Coronary Artery Surgery Study (CASS), detailed coding of coronary angiograms was available in 24,959 patients. Of these patients, 73 (0.3%) had major coronary artery anomalies: 70 had one coronary anomaly and 3 had two coronary anomalies. The most common anomaly involved the circumflex coronary artery (60%). In 69% of these, the circumflex artery arose from a separate ostium in the right coronary sinus, and in 31% it originated as a branch of the right coronary artery. The most common anomalous course was anterior or posterior to the great vessels but not between the great vessels. The major exception to this finding was an anomalous right coronary artery; 7 of 15 such arteries coursed between the great vessels. Anomalous circumflex coronary arteries had a significantly greater degree of stenosis than that in nonanomalous arteries in age- and gender-matched control patients (p = 0.02). Despite this difference, at 7 years there was no significant difference in survival by location or degree of stenosis in the anomalous artery.
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            Anomalous coronary arteries: angiographic findings in 80 patients.

            Among 13010 adults who underwent coronary arteriography, 80 (0.61%) patients had a total of 83 anomalous coronary arteries. Thirty-three (41%) of the patients were of Hispanic origin, while out of the entire population studied 30% were Hispanic. The right coronary artery was the most common anomalous vessel. It was identified in 50 (62%) patients, arising in 35 from the left aortic sinus, in 14 from the posterior sinus, and in 1 from the left coronary artery. An anomalous circumflex artery was recognized in 22 (27%) patients. Nine (11%) patients presented an anomalous left anterior descending artery, 1 patient an anomalous left main coronary artery, and another an anomalous septal perforator artery. Twenty-three (29%) patients had concomitant congenital heart abnormalities, most commonly. bicuspid aortic valve and mitral valve prolapse. In each of 5 patients with complex congenital heart disease the course of the anomalous vessel could have interfered with a surgical procedure. In 4 cases anomalous coronary arteries were associated with either anomalous systemic venous circulation or anomalous cardiac veins. In 5 (6%) patients only, the anomalous coronary artery was solely responsible for a clinical event. Coronary atherosclerosis of the anomalous arteries was found in 28% of the patients, while the overall incidence of the disease in this series was 65%. Thus, anomalous coronary arteries are associated with a high incidence of congenital heart diseases, but do not appear to be associated with an increased risk for development of coronary atherosclerosis. The angiographic recognition of these vessels is important in patients who undergo coronary angioplasty or cardiac surgery. Variations in the frequency of congenital coronary anomalies as reported herein may be attributed to a genetic background.
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              The four subtypes of anomalous origin of the left main coronary artery from the right aortic sinus (or from the right coronary artery).


                Author and article information

                S. Karger AG
                March 2007
                31 August 2006
                : 107
                : 3
                : 209-212
                Second Department of Cardiology, Interventional Unit, Evangelismos General Hospital, Athens, Greece
                95419 Cardiology 2007;107:209–212
                © 2007 S. Karger AG, Basel

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                Figures: 3, Tables: 1, References: 18, Pages: 4
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