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Clinical Articles: Coronary Artery Anomalies—Current Clinical Issues: Definitions, Classification, Incidence, Clinical Relevance, and Treatment Guidelines

Department of Adult Cardiology, Texas Heart Institute at St. Luke's Episcopal Hospital, Houston, Texas 77030, USA.
Texas Heart Institute journal / from the Texas Heart Institute of St. Luke's Episcopal Hospital, Texas Children's Hospital (Impact Factor: 0.63). 02/2002; 29(4):271-8.
Source: PubMed

ABSTRACT The study of coronary artery anomalies would benefit from the clarification of various fundamental issues, including the definitions, classification, incidence, pathophysiologic mechanisms, and clinical relevance of each anomaly. The greatest challenge is to identify the abnormality and determine its clinical relevance so that appropriate treatment can be instituted. Currently, the coronary anatomy is essentially defined by the features of the (conductive) epicardial coronary tree and its dependent territory. Therefore, one must consider all the possible and observed variations in anatomic features that are used to describe the coronary arteries. We propose that the left anterior descending, circumflex, and right coronary arteries be considered the essential elementary units of coronary anatomy. We also suggest that the coronary arteries be defined not by their origin or proximal course, but by their intermediate and distal segments or dependent microvascular bed. A strict classification system is necessary before meaningful data can be gathered about the incidence of coronary anomalies. With respect to clinical relevance, the greatest challenge is presented by anomalies that only occasionally cause critically severe clinical events and are otherwise compatible with a normal life. In such cases, it is not known whether the specific features of a given anomaly cause adverse clinical consequences, or whether additional episodic factors are required. To correlate subclassifiable anatomic and functional features with clinical events and prognoses, a large, multicenter database, relying on prospective, coordinated protocols, is urgently needed. In the absence of established official guidelines, we present practical protocols for diagnosing and treating coronary anomalies.

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    • "Documented coronary ischemia in the setting of an anomalous coronary artery coursing between aorta and pulmonary arteries is a class IB indication for surgery according to the American College of Cardiology and American Heart Association (ACC/AHA) guidelines for congenital heart diseases [12]. Exercise stress testing, though commonly employed for diagnosing coronary ischemia, is inadequate in predicting future risk of SCA in patients with anomalous coronaries [13] [14]. Guidelines also recommend surgical correction of anomalous coronary artery coursing between major vessels even in the absence of ischemia [12]. "
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    ABSTRACT: Sudden cardiac arrest has been reported to occur in patients with congenital anomalous coronary artery disease. About 80% of the anomalies are benign and incidental findings at the time of catheterization. We present a case of sudden cardiac arrest caused by anomalous left anterior descending artery. 61-year-old African American female was brought to the emergency department after sudden cardiac arrest. Initial EKG showed sinus rhythm with RBBB and LAFB with nonspecific ST-T wave changes. Coronary angiogram revealed no atherosclerotic disease. The left coronary artery was found to originate from the right coronary cusp. Cardiac CAT scan revealed similar findings with interarterial and intramural course. Patient received one-vessel arterial bypass graft to her anomalous coronary vessel along with a defibrillator for secondary prevention. Sudden cardiac arrest secondary to congenital anomalous coronary artery disease is characterized by insufficient coronary flow by the anomalous left coronary artery to meet elevated left ventricular (LV) myocardial demand. High risk defects include those involved with the proximal coronary artery or coursing of the anomalous artery between the aorta and pulmonary trunk. Per guidelines, our patient received one vessel bypass graft to her anomalous vessel. It is important for clinicians to recognize such presentations of anomalous coronary artery.
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    • "So far, no consensus exists to define and classify easily the wide spectrum of the congenital coronary artery abnormalities (Angelini, 2002). Numerous, sometimes long or complex, descriptions have been presented in the literature (Angelini 2007, Dodge-Khatami et al., 2000, Jacobs & Mavroudis, 2010, Rigatelli et al., 2009, Roberts, 1986). "
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    • "The anomalies with the highest risk of developing major cardiac events include coronary anomalous origin with interarterial course of the proximal segment, anomalous origin from the pulmonary artery, and the coronary arterovenous fistula [2] [3]. Other anomalies, such as anomalous origin from the wrong sinus or singular coronary artery without interarterial course, are generally benign, although not completely free of cardiac risk [4] [5] [6]. "
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    ABSTRACT: When the anomalous origin of coronary arteries (AOCA) is suspected in children (especially athletes), due to signs and symptoms of myocardial ischemia or on the basis of echocardiographic assessment, three-dimensional coronary magnetic resonance angiography (3D-CMRA) can be proposed for the fine morphological evaluation of coronary branches anatomy and course. We tested the diagnostic potential of CMRA angiography in a prospective study on AOCA in young patients. Between July 2005 and June 2008, 15 patients aged 6-29 years (mean age, 13.5 years+/-5.6 S.D.; median, 14) with clinical and echocardiographic suspicion of AOCA underwent CMRA (1.5 T), 3D whole-heart, free-breathing technique, without the use of contrast medium and beta-blockers, with a mean examination time of 30 min. We acquired a second scan of all patients to ameliorate the quality of the acquisition and to improve our experience. AOCA was confirmed by 3D-CMRA in 8 out of 15 cases (53%) and three different anatomical variants were demonstrated, that is, ectopic origin of the left circumflex artery arising from the right coronary artery with retro-aortic course in four cases, single coronary artery arising from the right sinus of Valsalva with interarterial course in one case, ectopic right coronary artery arising from the left sinus of Valsalva with interarterial course in one case; in two patients without anomalies of origin of the coronary arteries, elongated LMCA with angulation of the proximal segment of the left circumflex artery was present. When AOCA is suspected particularly in children (especially athletes), CMRA without the use of contrast medium is an effective diagnostic technique, which is useful to clarify the spatial position of the anomalous course of the main coronary branches in order to suggest the most convenient management of the disease. CMRA does not need contrast medium, needles, and beta-blockers; is repeatable in the same examination without the exposure to X-rays; allows a parent to stay near the child; and needs low collaboration in low-stress conditions.
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