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Congenital Coronary Artery Anomalies

Jack Wallen, MD, PhD, Alan O’Donnell, PA-C, James S. Tweddell, MD
Congenital Coronary Artery Anomalies is a topic covered in the Adult and Pediatric Cardiac.

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Introduction

Congenital coronary artery anomalies are rare occurring in 0.2-1.4% of the general population.[1],[2] Estimates on the prevalence of congenital coronary artery anomalies that are potentially pathologic, range from 0.1 to 1.0%.[1],[2],[3],[4],[5],[6],[7],[8]

These anomalies may involve abnormalities in the origin, course, or termination of the artery, as well as an abnormal number of coronary arteries. Coronary artery abnormalities may be acquired, as in cases of Kawasaki disease. The spectrum of coronary artery anomalies ranges from minor deviations in anatomy, such as a high coronary ostium, to potentially life-threatening anomalies such as anomalous origin of the left main coronary artery (LMCA) from the pulmonary artery, and anomalous aortic origin of a coronary artery with an intramural and interarterial course. Patients with congenital coronary artery anomalies may have a normal physical exam and can remain asymptomatic for many years. However, these anomalies can result in myocardial ischemia with angina, arrhythmias, and sudden cardiac death. Standard clinical tests, such as electrocardiogram (ECG) and cardiac enzymes, are unreliable in detecting these anomalies until flow abnormalities are severe. More advanced methodologies, including echocardiography and myocardial perfusion imaging, may detect these anomalies even in the absence of symptoms, but in the absence of symptoms, are not routinely performed. The potential for sudden death as the initial presentation makes it imperative that physicians be aware of the exercise/physical activity recommendations for patients with these anomalies.

This chapter will examine the following coronary anomalies: anomalous origins of coronary arteries from the pulmonary artery, anomalous aortic origin of a coronary artery from the aorta, coronary artery fistulas, and miscellaneous coronary anomalies. The epidemiology, anatomy, pathophysiology, and natural history of these anomalies will be described. Clinical management of these patients, including clinical presentation, diagnosis, medical and surgical management, will be discussed.

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Introduction

Congenital coronary artery anomalies are rare occurring in 0.2-1.4% of the general population.[1],[2] Estimates on the prevalence of congenital coronary artery anomalies that are potentially pathologic, range from 0.1 to 1.0%.[1],[2],[3],[4],[5],[6],[7],[8]

These anomalies may involve abnormalities in the origin, course, or termination of the artery, as well as an abnormal number of coronary arteries. Coronary artery abnormalities may be acquired, as in cases of Kawasaki disease. The spectrum of coronary artery anomalies ranges from minor deviations in anatomy, such as a high coronary ostium, to potentially life-threatening anomalies such as anomalous origin of the left main coronary artery (LMCA) from the pulmonary artery, and anomalous aortic origin of a coronary artery with an intramural and interarterial course. Patients with congenital coronary artery anomalies may have a normal physical exam and can remain asymptomatic for many years. However, these anomalies can result in myocardial ischemia with angina, arrhythmias, and sudden cardiac death. Standard clinical tests, such as electrocardiogram (ECG) and cardiac enzymes, are unreliable in detecting these anomalies until flow abnormalities are severe. More advanced methodologies, including echocardiography and myocardial perfusion imaging, may detect these anomalies even in the absence of symptoms, but in the absence of symptoms, are not routinely performed. The potential for sudden death as the initial presentation makes it imperative that physicians be aware of the exercise/physical activity recommendations for patients with these anomalies.

This chapter will examine the following coronary anomalies: anomalous origins of coronary arteries from the pulmonary artery, anomalous aortic origin of a coronary artery from the aorta, coronary artery fistulas, and miscellaneous coronary anomalies. The epidemiology, anatomy, pathophysiology, and natural history of these anomalies will be described. Clinical management of these patients, including clinical presentation, diagnosis, medical and surgical management, will be discussed.

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Last updated: March 31, 2022