Abstract
Coronary arteries may be anomalous, with an incidence of 0.17% at autopsy or 1.2% at coronary angiography. They may be benign, but also a cause of congestive heart failure or sudden arrhythmic cardiac death. In the Cardiac Registry of the Congenital Heart Diseases of the Institute of Pathological Anatomy (1700 specimens) in Padua/Italy, isolated malformations of the coronary arteries account for 3.5% of observed alterations. In this chapter, coronary artery anomalies as a cause of sudden death in athletes will be treated. Among 75 cases of sudden death during sport activity, 16% were attributed to coronary artery anomalies:
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1.
Origin of a coronary artery from opposite wrong aortic sinus
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Anomalous origin of the left circumflex coronary artery from the right sinus or coronary artery, with a retroaortic course.
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3.
High take-off
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Myocardial bridging.
Only origin from the pulmonary artery is considered a certain cause, whereas anomalous origin of the left coronary artery from the right sinus with interarterial course should be considered as highly probable cause. High take-off, right coronary artery from the left sinus and myocardial bridging remain uncertain. Myocardial bridging is quite frequent in hypertrophic cardiomyopathy and may be a cause of myocardial infarction-ischemic scarring; however, sudden death in hypertrophic cardiomyopathy may also occur in the absence of myocardial bridging. Oddly enough, sudden death cases with myocardial bridging reported in the literature were indeed hypertrophic cardiomyopathy cases.
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Clinical Cases
Clinical Cases
1.1 Questions
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A 53-year-old man was admitted to the coronary care unit complaining of chest pain. ECG showed ST-segment elevation in leads II, III, aVF, V5 and V6 as well as a ST-segment depression in V1-V4. Echocardiogram showed posterolateral left ventricular wall akinesia with preserved ejection fraction. There was an increase of the serum level of troponin. The patient was found dead 1 month later. At autopsy, no signs of obstructive coronary atherosclerotic disease were found. The left circumflex coronary artery took off at an acute angle from the anterior right aortic sinus, separately from the right coronary ostium, and run behind the aorta to reach the left atrioventricular groove. Moreover, a myocardial infarction was detected in the posterolateral wall of the left ventricle. Which imaging testing do you perform in this man to rule out coronary artery disease? In the guidelines of autopsy of sudden death, is this anomaly considered as a certain, a probable or an uncertain cause of death?
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A 3-month-old male baby was found dead in his crib. At autopsy, the origin of the dominant right coronary artery was found originating from above the left coronary sinus with an acute angle take-off, and the proximal segment coursed intramurally within the aortic tunica media, passing between the aorta and the pulmonary trunk before reaching the right atrioventricular groove. Could a fetal echocardiography provide the correct diagnosis of this condition?
1.2 Answers
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Coronary angiography is the gold standard method to provide an accurate study in vivo of coronary anatomy, to look at the origin and course of the proximal segments of right and left coronary artery, but also to rule out atherosclerotic coronary artery disease. Computed tomography angiography, due to its non-invasiveness and multiplanar reconstruction, is increasingly utilized for characterization of coronary artery origin anomalies. It provides a more accurate evaluation of the ostium as well as of the course and the presence of calcifications. Diagnostic methods such as computed tomographic angiography, nuclear magnetic resonance or coronarography are characterized by higher sensitivity and specificity as compared to echocardiography.
The anomaly is considered an uncertain cause of sudden death; however, the subendocardial healed myocardial infarction in the setting of patently related coronary arteries strongly support a causal relationship.
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Prenatal echocardiography does not allow visualization of the coronary artery origins in foetuses.
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Thiene, G., Rizzo, S., Frescura, C., Basso, C. (2020). Specific Cardiovascular Diseases and Competitive Sports Participation: Coronary Anomalies and Myocardial Bridging at Risk of Sudden Death. In: Pressler, A., Niebauer, J. (eds) Textbook of Sports and Exercise Cardiology. Springer, Cham. https://doi.org/10.1007/978-3-030-35374-2_21
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