Abstract
Anomalies of the aortic arch and the brachiocephalic vessels may occur in isolation or in association with congenital heart disease. Echocardiographic assessment of possible abnormalities in the position or branching of the aortic arch requires a systematic approach, based on the knowledge of the hypothetic bilateral aortic arch model according to Edwards. Identification of the brachiocephalic vessels and their connections is greatly enhanced by colour Doppler interrogation. Examination starts with the determination of the first brachiocephalic vessel and the position of the aorta from a high parasternal or suprasternal window. This approach allows to discriminate right, left and double aortic arch. In patients with double aortic arch, colour Doppler reveals patency or obstruction of either of the aortic arches. The presence of the thymus and the good imaging conditions in newborns and young infants allow to diagnose anomalies of the subclavian arteries and carotid arteries. These anomalies include aberrant origin of the subclavian artery from the descending aorta, isolation or cervical origin. Since determination of the laterality of the aortic arch and exclusion of pulmonary artery sling can be achieved quickly in infants, echocardiography represents an ideal screening method for detection of the majority of cardiovascular anomalies, which may cause obstructions of the upper airways in infancy.
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23.1 Electronic Supplementary Material
Video 23.1
This high parasternal short-axis sweep shows evaluation of laterality in a patient with normal left aortic arch. It starts with the parasternal short-axis view of the pulmonary artery bifurcation, displaying the ascending aorta in cross section. Cranial tilt of the transducer shows the transverse aortic arch, the superior vena cava in cross section and the innominate vein in longitudinal section. Further cranial tilt displays the origin of the innominate artery from the aorta to the right. Following clockwise rotation of the transducer combined with some more cranial tilt, the innominate artery is depicted in longitudinal section revealing its bifurcation into the right subclavian and right carotid artery (AVI 49571 kb)
Video 23.2
Colour Doppler in this high parasternal short-axis sweep shows evaluation of laterality in a patient with normal left aortic arch in analogy to Video 23.2 (AVI 23211 kb)
Video 23.3
Slight rotation of the transducer from the long-axis view of the innominate artery displays the branching into right subclavian and right common carotid artery (AVI 5470 kb)
Video 23.4
The high parasternal short-axis view in a patient with right aortic arch shows origin of the first brachiocephalic artery to the left. Branching of the innominate artery into left subclavian and left common carotid artery characterizes this vessel as left innominate artery (AVI 5812 kb)
Video 23.5
The anatomy is confirmed by colour Doppler interrogation displaying the innominate vein cranial to the aorta and the superior vena cava to the right of the aorta (AVI 5868 kb)
Video 23.6
This high parasternal short-axis sweep shows evaluation of laterality in a patient with right aortic arch. It starts with the parasternal short-axis view displaying the ascending aorta in cross section. Cranial tilt of the transducer shows the superior vena cava in cross section and the innominate vein in longitudinal section. Further cranial tilt displays the origin of the innominate artery from the aorta to the left branching into the left subclavian and left common carotid artery (AVI 23620 kb)
Video 23.7
Colour Doppler in this high parasternal short-axis sweep shows evaluation of laterality in a patient with right aortic arch. It starts with the parasternal short-axis view of the pulmonary artery bifurcation, displaying the ascending aorta in cross section. Cranial tilt of the transducer shows the superior vena cava in cross section and the innominate vein in longitudinal section. Clockwise rotation and some rightward tilt of the transducer open the aortic arch and display the right descending aorta (AVI 22931 kb)
Video 23.8
The colour Doppler sweep in a young infant with double aortic arch starts in the high parasternal short-axis view displaying the ascending aorta in cross section. Cranial tilt of the transducer displays the innominate vein and shows origin of both aortic arches from the ascending aorta with transition of the circular shape of the aorta into the configuration of a horizontal 8. Further cranial tilt shows separation of the right and left aortic arch. Colour Doppler reveals patency both of the right and left aortic arches (AVI 16269 kb)
Video 23.9
The colour Doppler sweep in another infant with double aortic arch starts in the high parasternal short-axis view displaying the ascending aorta in cross section. Cranial tilt displays the innominate vein cranial to the aorta and shows separation of the aorta into a dominant right and a significantly smaller left aortic arch. Further cranial tilt of the transducer confirms patency of both aortic arches (AVI 29467 kb)
Video 23.10
Colour Doppler in a cranial short-axis view (same patient as in Video 23.8) displays the complete vascular ring formed by a patent right and left aortic arch (AVI 2392 kb)
Video 23.11
The colour Doppler sweep in a high parasternal long-axis view in a patient with double aortic arch starts with a long-axis view of the right aortic arch. Leftward tilt of the transducer shows the longitudinal view of the oesophagus; further leftward tilt of the transducer reveals the left aortic arch which reveals a smaller segment distal to the left carotid artery. Distal to the left subclavian artery, the aorta is larger due to the ampulla of the former ductus arteriosus. Finally, the transducer is tilted back towards the right displaying again the larger right aortic arch (AVI 40941 kb)
Video 23.12
In a newborn with double aortic arch, the left aortic arch is displayed from a suprasternal window revealing atresia distal to the origin of the left carotid and left subclavian artery. Absence of antegrade flow across the aortic isthmus as well as retrograde filling (coded in red) of the distal left descending aorta (diverticulum of Kommerell) from the right aortic arch is evident on colour Doppler examination (AVI 1866 kb)
Video 23.13
This colour Doppler sweep in the high left parasternal sagittal view (ductal view) starts with a lateral plane displaying the main and left pulmonary artery (same patient as in Video 23.12). Leftward tilt of the transducer reveals a small and restrictive ductus arteriosus connecting to the MPA just proximal of the origin of the LPA. The ductus originates from the distal left descending aorta. Further rightward tilt displays the left aortic arch interrupted distal to the left subclavian artery, while the distal left aortic arch is filled retrogradely. Finally, the transducer is tilted again to the left (WMV 13225 kb)
Video 23.14
The high left parasternal short-axis view in a 4-month-old infant shows a left innominate artery dividing into the left carotid and left subclavian artery suggesting a right aortic arch. However, the left subclavian artery shows an inferior angulation, indicating the correct diagnosis of double aortic arch with partial atresia of the left arch distal to the left subclavian artery (AVI 6111 kb)
Video 23.15
Colour Doppler confirms regular flow in the innominate artery and branching into left common carotid and left subclavian artery (same patient as in Video 23.14) (AVI 1381 kb)
Video 23.16
The suprasternal long-axis view in a newborn shows a right aortic arch, giving origin to the right carotid and right subclavian artery. The view was obtained by clockwise rotation of the transducer from the suprasternal short-axis view (same patient as in Videos 23.17 and 23.18) (AVI 3016 kb)
Video 23.17
The oblique high left parasternal short-axis view (same patient as in Videos 23.16 and 23.18) displays the ascending aorta in cross section giving rise to the left common carotid artery originating as the first brachiocephalic vessel. The left subclavian artery originates as the last brachiocephalic vessel from a diverticulum of Kommerell, which represents the distal portion of the left aortic arch. From the top of the diverticulum of Kommerell originates the left-sided ductus arteriosus which is further delineated in the sweep of Video 23.18 (AVI 1040 kb)
Video 23.18
The colour Doppler sweep in this newborn reveals the diagnosis of RAA, aberrant left subclavian artery and left ductus arteriosus originating from a diverticulum of Kommerell (same patient as in Videos 23.16 and 23.17). It starts in the oblique high left parasternal short-axis view displaying aberrant origin of the left subclavian artery from a diverticulum of Kommerell. Clockwise rotation of the transducer and caudal shift reveal a tortuous ductus arteriosus originating from the top of the diverticulum of Kommerell connecting to the pulmonary bifurcation (AVI 19067 kb)
Video 23.19
The oblique suprasternal short-axis view in a 4-year-old patient with right aortic arch shows the ascending aorta in cross section giving rise to the left common carotid artery, which originates as the first brachiocephalic vessel (same patient as in Video 23.20). The left subclavian artery originates aberrantly from the descending aorta via a diverticulum of Kommerell (AVI 5371 kb)
Video 23.20
Separate origin of the left carotid and left subclavian artery is confirmed by colour Doppler (same patient as in Video 23.19). Colour Doppler reveals no evidence for persistent patency of the ductus arteriosus (AVI 1824 kb)
Video 23.21
The high right parasternal short-axis view in a neonate with left aortic arch shows the ascending aorta (AO) in cross section giving rise to a brachiocephalic vessel to the right. The rather small size of this first vessel suggests that it might not represent the right innominate artery but only the right common carotid artery (AVI 17226 kb)
Video 23.22
Caudal tilt of the transducer in a similar patient depicts the right subclavian artery, taking a parallel but inferior course to the carotid artery, due to its aberrant origin from the descending aorta (AVI 3286 kb)
Video 23.23
Caption (AVI 2620 kb)
Video 23.24
Colour Doppler in a posterior suprasternal short-axis plane of a newborn with left aortic arch shows origin of the aberrant right subclavian artery from the descending aorta. Note significant retrograde flow in the descending aorta in diastole, which is explained by the diagnosis of truncus arteriosus resulting in diastolic run-off to the pulmonary arteries (AVI 14408 kb)
Video 23.25
In an infant with pulmonary atresia, VSD and left aortic arch, colour Doppler in the longitudinal view of the right neck shows the right common carotid artery. Proximal to the bifurcation into internal and external carotid artery, the right subclavian artery is displayed taking a cervical origin (same patient as in Videos 23.26 and 23.27 (AVI 2870 kb)
Video 23.26
Caudal angulation of the transducer displaying the right brachiocephalic vessels in the lower region of the right neck shows the caudal course of the right subclavian artery parallel to the right common carotid artery (same patient as in Videos 23.25 and 23.27) (AVI 1901 kb)
Video 23.27
Caudally directed flow in the subclavian artery as opposed to cranial flow in the common carotid artery is confirmed by colour Doppler (same patient as in Videos 23.25 and 23.26) (AVI 2681 kb)
Video 23.28
The oblique suprasternal short-axis view in a newborn with right aortic arch shows the ascending aorta in cross section giving rise to the left common carotid artery, which originates as the first brachiocephalic vessel. In a more caudal position, a diverticulum of Kommerell is visualized with some distance to the ascending aorta. Colour Doppler fails to demonstrate antegrade flow from the diverticulum to the left subclavian artery. Instead colour Doppler reveals retrograde flow in the left subclavian artery suggesting perfusion from the left vertebral artery and confirming the rare diagnosis of isolation (AVI 1574 kb)
Video 23.29
Colour Doppler from a right cervical window in a newborn with left cervical aortic arch shows cranial extension and tortuosity of the aortic arch (AVI 4140 kb)
Video 23.30
Colour Doppler in the parasternal short-axis view of a normal newborn shows the pulmonary bifurcation with normal origin of right and left pulmonary artery (AVI 2697 kb)
Video 23.31
The suprasternal short-axis view in a newborn with pulmonary artery sling shows the aorta in cross section and the right pulmonary artery in longitudinal section. The left pulmonary artery takes a distal origin from the right pulmonary artery with a retrograde course to the left hilum (same patient as in Video 23.32) (AVI 25625 kb)
Video 23.32
Colour Doppler in the suprasternal short-axis view of this newborn (same patient as in Video 23.31) confirms distal origin of the left pulmonary artery. Colour Doppler reveals proximal diastolic inflow into the main pulmonary artery originating from a small ductus arteriosus at the expected site of origin of the left pulmonary artery from the bifurcation (AVI 6343 kb)
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Hofbeck, M., Deeg, KH., Rupprecht, T. (2017). Vascular Rings and Anomalies of the Aortic Arch Vessels. In: Doppler Echocardiography in Infancy and Childhood. Springer, Cham. https://doi.org/10.1007/978-3-319-42919-9_23
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DOI: https://doi.org/10.1007/978-3-319-42919-9_23
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