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Congenital Coronary Artery Anomalies

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Transesophageal Echocardiography for Congenital Heart Disease

Abstract

Assessment of coronary artery anatomy has become an important component of the echocardiographic examination in many forms of congenital heart disease, however the presence of isolated congenital or acquired coronary artery anomalies in an otherwise structurally normal heart has only recently been appreciated. Because of its superior resolution, transesophageal echocardiography (TEE) is recognized as an important diagnostic tool to image coronary artery anomalies. This chapter will focus on the anatomic features and TEE evaluation of isolated congenital coronary anomalies. In addition, this chapter also provides a brief discussion in the use of TEE in patients with Kawasaki disease who have important coronary artery involvement.

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Authors and Affiliations

  1. Division of Pediatric Cardiology, Department of Pediatrics, Medical College of Wisconsin, Children’s Hospital of Wisconsin, 9000 W. Wisconsin Avenue, Milwaukee, WI, 53122, USA

    Peter C. Frommelt MD

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  1. Peter C. Frommelt MD
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Correspondence to Peter C. Frommelt MD .

Editor information

Editors and Affiliations

  1. Childrens Hospital Los Angeles Department of Cardiology, University of Southern California Keck School of Medicine, Los Angeles, California, USA

    Pierre C. Wong

  2. Pediatric Cardiovascular Anesthesiology, Texas Children's Hospital, Houston, USA

    Wanda C. Miller-Hance

Electronic Supplementary Material

Below is the link to the electronic supplementary material.

Transesophageal echocardiogram from a midesophageal aortic valve short-axis window through the aortic root in a patient with anomalous origin of the right coronary artery from the left sinus of Valsalva and an intramural course of the anomalous vessel. The anomalous right coronary artery can be seen arising from the left sinus of Valsalva (L) and coursing intramurally within the anterior aortic wall between the aorta and the right ventricular outflow tract before exiting the aortic wall from the right sinus of Valsalva (R). The non-coronary cusp (N) is seen posteriorly (MOV 7690 kb)

Video 14.1b

Video is color Doppler imaging in the same patient in Video 14.1a. Color Doppler imaging shows the linear diastolic flow of the anomalous coronary within the anterior aortic wall; the blue color signal confirms anomalous coronary flow away from the transducer anteriorly, consistent with the coronary originating from the left sinus and coursing towards the more anteriorly positioned right sinus. The Nyquist limit has been lowered to 28 cm/s to enhance visualization of the low-velocity diastolic coronary flow. The multiplane transducer has been rotated to an angle of 33° to optimize imaging of the anomalous coronary origin. The left coronary artery origin is not visualized (MOV 1841 kb)

Midesophageal aortic valve short-axis view through the aortic root in a patient with anomalous origin of the right coronary artery from the left sinus of Valsalva and short intramural course of the anomalous coronary. The relationship between the aortic intercoronary commissure and the anomalous right coronary artery origin can be seen in this sweep with the coronary arising from the rightward aspect of the left sinus of Valsalva near the commissure with a short intramural course before exiting the aortic wall from the right sinus of Valsalva. The short intramural course with the anomalous origin of the right coronary near the aortic commissure is typical of this lesion. The multiplane transducer has been angled at 33° to optimize imaging of the anomalous coronary origin. The left coronary origin is not visualized (MOV 38178 kb)

Midesophageal aortic valve short-axis view; imaging clip through the aortic root in a patient with anomalous origin of the left coronary artery from the right sinus of Valsalva. The relationship between the aortic commissure and the anomalous left coronary artery origin can be seen in this sweep with the coronary arising from the mid-portion of the right sinus of Valsalva with a long intramural course within the anterior aortic wall before exiting the wall from the left sinus of Valsalva. The longer intramural course with the anomalous origin of the left coronary from the middle of the right sinus is typical of this lesion, in contrast to the shorter intramural course and origin adjacent to the commissure with anomalous origin of the right coronary from the left sinus (as shown in Video 14.2). The multiplane transducer has been angled at 67° to optimize imaging of the anomalous coronary origin. The right coronary origin is not visualized (MOV 7535 kb)

Midesophageal aortic valve short-axis view imaging clip through the aortic root in the same patient shown in Video 14.3 with anomalous origin of the left coronary artery from the right sinus of Valsalva. The imaging plane is angled slightly superiorly to better visualize the origin of both the anomalous left coronary and the normally positioned right coronary as both arise from the right sinus of Valsalva. The long intramural course of the anomalous left coronary within the anterior aortic wall is well visualized (MOV 7373 kb)

Midesophageal aortic valve short-axis view color Doppler clip through the aortic root in the same patient shown in Video 14.3 with anomalous origin of the left coronary artery from the right sinus of Valsalva demonstrates the red linear diastolic flow signal in the intramural segment coursing posteriorly towards the left sinus (and the posteriorly positioned TEE transducer). The multiplane transducer again has been rotated to an angle of 67° to optimize imaging of the anomalous coronary origin (MOV 5478 kb)

Midesophageal long-axis clip at 128° through the left ventricle in a patient with anomalous origin of a coronary from the opposite sinus with an interarterial course. The anomalous coronary is seen coursing anterior to the aorta between the great arteries as a discreet circle; the probe is then rotated towards the patient’s left (counterclockwise) to follow the course of the left coronary as the intramural portion of the proximal left coronary exits the aortic wall and bifurcates into the left anterior descending and circumflex coronary branches (MOV 41933 kb)

Transesophageal imaging sweep from a deep transgastric long-axis position through the left ventricle and aortic outflow in a plane of 0° from a patient with anomalous origin of the left coronary artery from the right sinus of Valsalva with an intramural course. As the imaging plane is angled more anteriorly towards the right ventricular outflow tract, the intramural segment of the left coronary and normal course of the right coronary along the anterior aspect of the aortic root is well imaged. The usual origin of the left coronary from the left sinus, which is frequently well imaged from this view, is not seen (MOV 37437 kb)

Video 14.7

Midesophageal aortic valve short-axis imaging clip through the aortic root in the same patient shown in Videos 14.3, 14.4a, and 14.4b with anomalous origin of the left coronary artery from the right sinus of Valsalva after surgical unroofing. The long intramural course of the anomalous left coronary within the anterior aortic wall has been removed, and the coronary now appears to arise normally from the left sinus of Valsalva, with an easily visualized neo-orifice from the appropriate sinus (MOV 5038 kb)

Video 14.8a

Midesophageal aortic valve short-axis TEE image clips of the anomalous insertion of the left main coronary artery into the posterior aspect of the proximal main pulmonary artery (PA) in a patient with anomalous left coronary artery from the pulmonary artery (ALCAPA). The left coronary is seen connecting to the pulmonary artery through a short left main coronary segment and then bifurcating into the left anterior descending and circumflex branches (arrows), clearly a long distance from the normal entrance into the aortic root (Ao). (MOV 2109 kb)

Video 14.8b

Video is color Doppler imaging in the same patient in Video 14.8a Color Doppler shows a blue color signal in the left coronary artery (arrows), consistent with coronary flow towards the pulmonary artery, and there is a prominent color Doppler signal in the main pulmonary artery as the retrograde flow of the anomalous coronary empties into the pulmonary artery (MOV 1369 kb)

Video 14.9

Midesophageal aortic valve short-axis TEE imaging clip of a dilated left coronary artery (LCA) in a child with anomalous right coronary artery from the pulmonary artery; the left coronary artery measured 3.5 mm at its origin, which is significantly abnormal for age. The left coronary arises normally from the aortic root (Ao); the anomalous right coronary is not seen. The left coronary is dilated because of the increased flow into the left coronary bed as it functions as the single coronary supplying the entire myocardium with steal of coronary flow from the anomalous right coronary emptying into the low pressure pulmonary bed (MOV 3850 kb)

Midesophageal right ventricular inflow-outflow imaging clip at 52° using color Doppler to identify the course of the anomalous coronary in the same patient as Video 14.9 with anomalous right coronary artery from the pulmonary artery. The coronary is imaged anterior to the right aortic sinus of Valsalva taking a tortuous course (arrows) with the color Doppler signal demonstrating flow towards the main pulmonary artery. The coronary artery appears remote from its usual origin off the right aortic sinus (MOV 2760 kb)

Midesophageal long-axis imaging clip at 101° through the left ventricle (LV) in the same patient as Videos 14.9 and 14.10 with anomalous origin of the right coronary from the pulmonary artery (ARCAPA). There is a continuous color Doppler flow signal entering the pulmonary artery (PA) anteriorly above the right ventricular outflow tract (RVOT). A diastolic flow signal in the PA is seen because the anomalous coronary is supplied retrograde from the normally arising left coronary. The retrograde flow empties into the pulmonary artery because of the lower pressure in the PA. This coronary steal flow signal may be the first clue that there is an anomalous coronary artery arising from the PA, as direct visualization of a coronary entering the PA can sometimes be difficult to obtain (MOV 4082 kb)

Video 14.12

Transgastric mid short-axis imaging clip at −10° through the right ventricular apex from a patient with a left coronary artery to right ventricular fistula. This video shows color Doppler imaging of the fistula as it empties into the right ventricle near the apex (MOV 3784 kb)

Video 14.13a

Midesophageal aortic valve short-axis view at 0° of the aorta (Ao) displaying origin of the right coronary artery (RCA) in a patient with a right coronary artery fistula. The RCA arises appropriately from the anterior aspect of the aorta and is markedly dilated (arrows) because of the increased flow into that vessel, which measured 5 mm in diameter proximally. (MOV 2419 kb)

Video 14.13b

Video is color Doppler imaging in the same patient in Video 14.13a. The continuous flow signal is easily appreciated by color Doppler without decreasing the Nyquist scale because of the high volume flow into the coronary artery from the fistula steal (MOV 1951 kb)

Video 14.14a

Transesophageal imaging from a deep transgastric sagittal position through the left ventricle (LV) and aortic outflow (Ao) in a longitudinal plane at 90° from the same patient shown in Video 14.12 with a right coronary artery fistula. Because of the increased flow into the right coronary artery (RCA) to supply the fistula, the proximal right coronary is easily seen by two-dimensional imaging and appears markedly dilated (arrows). The right ventricle is seen anteriorly (RV) and appears dilated, secondary to the fistula shunt. (MOV 2713 kb)

Video 14.14b

Transesophageal imaging from a deep transgastric sagittal position through the left ventricle (LV) and aortic outflow (Ao) in a longitudinal plane at 90° from the same patient shown in Video 14.12 with a right coronary artery fistula. With color Doppler continuous flow into the distal right coronary is visualized, again reflecting the increased flow into the coronary artery secondary to the fistula steal. The distal termination site of the fistula into the right ventricle is not visualized in this image (MOV 2773 kb)

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© 2014 Springer-Verlag London

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Frommelt, P.C. (2014). Congenital Coronary Artery Anomalies. In: Wong, P., Miller-Hance, W. (eds) Transesophageal Echocardiography for Congenital Heart Disease. Springer, London. https://doi.org/10.1007/978-1-84800-064-3_14

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  • DOI: https://doi.org/10.1007/978-1-84800-064-3_14

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