Methodology of echocardiographic analysis of morphological variations of the aortic arch and its branches in children – own experience

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Polish Ultrasound Society (Polskie Towarzystwo Ultrasonograficzne)

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VOLUME 19 , ISSUE 76 (Apr 2019) > List of articles

Methodology of echocardiographic analysis of morphological variations of the aortic arch and its branches in children – own experience

Wojciech Mądry / Ewa Zacharska-Kokot / Maciej A. Karolczak *

Keywords : echocardiography, right-sided aortic arch, double aortic arch, vascular ring, aberrant subclavian artery

Citation Information : Journal of Ultrasonography. Volume 19, Issue 76, Pages 24-42, DOI: https://doi.org/10.15557/JoU.2019.0004

License : (CC-BY-SA-4.0)

Received Date : 04-November-2018 / Accepted: 22-January-2019 / Published Online: 30-April-2019

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FIGURES & TABLES

Fig. 1.

Ultrasound sections showing the thoracic aorta with left aortic arch in frontal and transverse planes. The images were obtained by gradually tilting the ultrasound beam (scan) from the frontal plane posteriorly and upwards, until reaching a horizontal plane: A. High para/suprasternal transverse view in a frontal-like plane. Centrally located ascending aorta is directed with its small convexity to the right; the upward flow is coded red. On the left – a transverse section of the pulmonary artery trunk (PA); below – the left atrium (LA) with visible orifices of the right upper (RUPV) and the left inferior pulmonary vein (LIPV) and the left atrial appendage (app). The left inferior pulmonary vein is posteriorly crossed by the descending aorta. B. A backward tilt of the ultrasound beam – to an intermediate plane between the frontal and horizontal planes. A transverse section of the upper segment of the ascending aorta (AoAsc) and, on its left, pulmonary artery trunk (PA) on the branching level and both branches (LPA and RPA) are visible. Superior vena cava (SVC) to the right of the aorta. The descending aorta (Desc) posteriorly crosses the left pulmonary artery at this level. C. Further, upward tilt of the ultrasound beam (up to the horizontal plane) visualizes the transverse part of the aortic arch (TrA), with its convexity characteristically directed to the left, slightly upwards and anteriorly; then it descends leftwards and posteriorly until reaching the anterior/left border of the thoracic spine. At this level, pulmonary arteries are no longer visible. D. Directing the ultrasound beam even higher shows branches of the arch, their typical arrangement and mutual proportions. For left aortic arch, the first branch is always directed to the right; in most cases this is the right brachiocephalic trunk (rBCT), clearly wider than the two vessels that follow, i.e. the left common carotid artery and the left subclavian artery; however, different anatomical variants are possible, including abnormalities, such as an aberrant right subclavian artery (ARSA). Visualization of the division of the first aortic arch branch into two equal arteries almost certainly excludes ARSA

Fig. 2.

A typical left aortic arch en face. A. Normal, left aortic arch en face. High parasternal view (the transducer is placed just below the suprasternal notch, slightly to the right). Obtaining clear images is possible owing to the presence of conductive thymus an incomplete ossification of thoracic bony structures in an infant. The ultrasound beam directed obliquely so as to simultaneously visualize the ascending aorta, the aortic arch and the descending aorta as well as the branches of the arch. The right brachiocephalic trunk, which is wider than the other branches, is the first branch. AoAsc – ascending aorta, TrA – transverse aortic arch, Isth – isthmus, Desc – descending aorta, RPA – right pulmonary artery, LA – left atrium, LMB – acoustic shadow of the main left bronchus, RBCT – brachiocephalic trunk, LCCA – left common carotid artery, LSA – left subclavian artery. B. An analogous image with color-coded flow. Legends as in Fig. 1A

Fig. 3.

Thoracic aorta in a patient with left aortic arch in sagittal-like parasternal views. The transducer located in the left parasternal line, slightly lower than in Fig. 1A and 1B (right-to-left scan). A. Ultrasound beam directed slightly rightwards, showing the ascending aorta (AoAsc) with the bulb. The right pulmonary artery (RPA) passes immediately behind the aorta. Other visible elements: left atrium (LA), right atrium with a fragment of its appendage (RA), an esophageal and tracheal shadow (Tr). B. A slightly more medial course of the ultrasound beam visualizes the initial segment of the transverse aortic arch and the initial segment of the brachiocephalic trunk (RBCT). Other legends as in Fig. 2A. C. A slight tilt of the transducer to the left of the medial plane with minimal rotation to the left visualizes the transverse aortic arch (TrA) and the initial segment of the descending aorta – the isthmus (Isth), as well as the pulmonary artery trunk (PA) and the initial segment of its left branch (LPA), which anteriorly crosses the descending aorta. D. A further slight tilt of the ultrasound beam to the left visualizes almost the entire descending aorta (Desc), as well as the distal segment of the aortic arch (TrA), the pulmonary artery (PA), the left upper pulmonary vein (LUPV) and the left ventricle (LV). The color-coded stream in the descending aorta is apparently interrupted at the level of the upper wall of the left atrium by an acoustic shadow produced by the left main bronchus (LMB), which anteriorly crosses the aorta and is located between the transducer and the aorta. The characteristic course of the individual aortic segments, which may be followed by performing maneuvers described in Fig. 2A, B and 3 A–D, is a decisive phenomenon for the assessment of aortic arch location and course. In the case of the left aortic arch, the aorta gradually travels to the front, rightwards and backwards, posteriorly crossing the left pulmonary artery. The characteristic convexity of the arch is directed to the left and upwards. The first branch, usually a wide brachiocephalic trunk, which divides after a short course, is directed to the right. In the case of the right aortic arch, the convexity of the arch is directed to the right, while the descending aorta remains on the right side of the chest and posteriorly crosses the right pulmonary artery, and the first branch is always directed to the left

Fig. 4.

Imaging of the aortic arch in the case of the left aortic arch with an aberrant right subclavian artery. A. High parasternal view showing the upper mediastinum in the horizontal plane analogous to the image in Fig. 2B. The right subclavian artery (ARSA) passing to the right, anteriorly and upwards, arises from the posteromedial surface of the aortic arch (TrA). The color disappears at the level of crossing with the esophagus (E) and trachea as the ultrasound beam is completely reflected by the trachea. The ARSA segment located right to the trachea is situated higher; hence its visualization in the same image is impossible. LIV – left innominate vein. B. The same image without color coding. Also, the ARSA segment located immediately behind the esophagus was not visualized. C. Suprasternal view showing the aortic arch en face. Typical left aortic arch forming three branches. The absence of differences in the diameters of the subsequent arteries is noticeable. RCCA – right common carotid artery, LCCA – left common carotid artery, LSA – left subclavian artery. D. The image in this figure is substantially the same as in Fig. 4 C. Apart from a slightly elongated aortic arch, minor disproportion in the diameters of branches (the first branch is wider than the two subsequent branches), which could suggest a typical pattern, is noticeable. E. An analysis of the course of the first branch of the aortic branch could suggest normal arterial anatomy. In reality, the right common carotid artery (RCCA) formed the vertebral artery (VA). Other legends as in Fig. 4 C. F. In a view showing the apex of the aortic arch (TrA), an abnormal origin of the right subclavian artery (*) TrA was visualized in the horizontal plane. G. Another variant possibly indicating normal pattern of aortic arch branches. A trunk formed by both common carotid arteries is the first branch, followed by the left subclavian artery, while the right subclavian artery (invisible in this view) is the last branch. AoAsc – ascending aorta, TrA – transverse aortic arch, RCCA – right common carotid artery, LCCA – left common carotid artery, LSA – left subclavian artery. H. In the case of difficulty visualizing the distal part of the aortic arch, it is usually possible to visualize the peripheral, abnormal segment of ARSA. High right horizontal suprasternal or parasternal view enables following the course of ARSA between the clavicle and the trachea; the proximal segment disappears behind ultrasound-impermeable trachea and esophagus (E). The right common carotid artery runs almost parallel and anteriorly to ARSA. Legends: ARSA – anomalous (aberrant) right subclavian artery, RCCA – right common carotid artery, LCCA – left common carotid artery, LSA – left subclavian artery. I. ARSA in a child with aortic coarctation. Increased narrowing of the aortic isthmus. This view is not suggestive of an additional vascular anomaly. TrA – transverse arch, * – coarctation, PA – pulmonary artery trunk, AoDesc – descending aorta, LSA – left subclavian artery. J. A two-dimensional image in a horizontal plane clearly showing the distal part of the aortic arch “dragged” to the right, and atypical dilation of this segment, as well as the initial part of ASRA (*). K. Color Doppler shows flow directed to the right, which disappears behind the trachea – an image typical of ARSA, but very difficult to interpret due to respiratory artifacts. Legends as in previous figures

Fig. 5.

Imaging of the left aortic arch with the right descending artery. The images were obtained by gradually tilting the ultrasound beam posteriorly and upwards from the frontal plane until reaching a horizontal plane. A. Suprasternal view – frontal plane. Systolic phase. The ascending aorta (AoAsc) is seen in the centre, with the left atrium (LA) located below. The transverse section of the descending aorta (AoDesc) is seen behind the right wall of the left atrium, posteriorly from the orifice of the right pulmonary veins (RPV). Furthermore, the pulmonary artery trunk (PA) is located left to the aorta; on the right side – the superior vena cava (SVC) and a part of the right pulmonary artery (RPA). It is noteworthy that the descending aorta is located exactly posteriorly from the superior vena cava – to the right from the midline – a phenomenon typical of right aortic arch. B. Ultrasound beam shifted slightly more posteriorly and horizontally. Legends as in previous figures. At this level, the descending aorta is adjacent to the posterior wall of the right pulmonary artery and is also located exactly posteriorly from the SVC. C. A slightly more horizontal plane – legends as in previous figures. The descending aorta is slightly closer to the ascending aorta, which is now seen in a transverse section. D. Continued upward scanning – the descending aorta is located just below the right pulmonary artery and is gradually approaching the ascending artery, still on the right side of the midline. E. A nearly horizontal plane. It is only at this level that the untypically coursing left pulmonary artery (LPA) directed posteriorly instead of leftwards in its initial course is visible. Although the descending aorta is closer to the ascending aorta, it is still located on its right side. A strongly hyperechoic trachea is located between the ascending and descending aorta. F. A section at the level of aortic arch (TrA). The convexity of the aortic arch is directed to the left, which is typical of the left aortic arch. G. A cross-section at the level of arch branches. The first branch is directed rightwards, the two other branches are directed to the left. This pattern points to a left aortic arch. A similar diameter of these vessels is noticeable (the first vessel and the two other vessels have similar diameters); therefore, the presence of an aberrant right subclavian artery is very likely. RRCA – right common carotid artery, LCCA – left common carotid artery, LSA – left subclavian artery. H. The first branch of the aortic arch in the frontal plane passes to the right and upwards; since no division within this vessel was visualized, this is the right common carotid artery (RCCA). A hyperechoic area, which could correspond to an aberrant right subclavian artery (marked [?]), is visible in the distal part of the aortic arch; however, none of the obtained images could clearly confirm this assumption (respiratory artifacts prevented Doppler flow assessment in this region)

Fig. 6.

Imaging of the right aortic arch. A. A suprasternal view, mediastinal section in the frontal plane. Diastolic phase. Elements visualized: ascending aorta (AoAsc), pulmonary artery trunk (PA), left atrium (LA). B. The view and legends as in Fig. 5A, a slight posterior tilt of the ultrasound beam, systole. Elements that are still visible: ascending aorta up to the apex of the transverse aortic arch, pulmonary trunk, the right pulmonary artery crossing the ascending aorta, with its division into upper lobar artery, middle artery, and the left atrium. C. Further posterior movement of the ultrasound beam no longer shows the aorta; the right pulmonary artery and the pulmonary trunk, as well as the transverse part of the aortic arch along with the first, leftward branch are still visible. It is difficult to decide in this view whether this is the left brachiocephalic trunk or the left subclavian artery. D. A definite backward tilting of the transducer shows the right aortic arch with its convexity directed to the right and the initial segment of the descending aorta. E. A wide, strong tracheal shadow significantly obscures the descending aorta; therefore, precise assessment of its lower segment in the image from the previous figure is not possible. Slight parallel movement of the transducer to the left visualizes both the longer segment of the first arch branch – a relatively narrow and arched upwards left common carotid artery (LCCA) – as well as the left subclavian artery (LSA) emerging from the tracheal acoustic shadow, initially wide and narrowing towards the periphery. The image is indicative of the presence of an aberrant left subclavian artery, arising from the descending aorta. F. Continued movement to the left allows following further course of the left branches of the aortic arch. LIV – left innominate vein

Fig. 7.

RAA with the brachiocephalic trunk forming the ductus arteriosus to LPA (most often the tetralogy of Fallot and pulmonary valve atresia with VSD). A. The view as in Fig. 5A (frontal plane). Systolic phase, the upward flow in the ascending aorta (AoAsc) is visible. B. Slight posterior movement of the ultrasound beam allows visualizing the right pulmonary artery with its division into upper lobar and middle artery, the transverse aortic arch at the site of first branch formation, as well as the middle segment of the descending aorta (Desc), which passes behind the left atrium (LA), posteriorly crossing two right pulmonary veins (RPV). C. Further posterior tilt of the ultrasound beam visualizes the transverse portion of the right aortic arch (AoA) with typical rightward convexity. Also, a small fragment of the left pulmonary artery (LPA) is visible. D. A slight movement of the transducer to the left is usually needed for a more detailed visualization of the first branch, which in this case is the left brachiocephalic trunk (LBCT). The left subclavian artery (LSA) runs on the left and slightly downwards from the trunk; visualization of the left common carotid artery often requires further movement and/or rotation of the transducer. E. A further slight movement of the transducer to the left allows to visualize LTBC division into LCCA and LSA. F. A newborn with pulmonary trunk atresia and VSD – ductus-dependent pulmonary circulation. Frontal-like plane section. Systole. The middle segment of the aortic arch (TrA), which forms a wide left brachiocephalic trunk (LTBC), which in turn gives rise to the left subclavian artery (LSA), is shown. The left common carotid artery is not visible in this figure. G. A slight posterior tilt of the transducer shows the initial segment of a tortuous ductus arteriosus (PDA) – which is too large fit in one plane

Fig. 8.

RAA with brachiocephalic trunk, the ductus arteriosus arising from the descending aorta and passing to LPA. A. A view showing the transverse portion of the aortic arch (TrA) with a clear rightward convexity. A very wide and short left brachiocephalic trunk dividing into a horizontal left subclavian artery (LSA) and the left common carotid artery (LCCA), which bends upwards. The distal part of the aortic arch is deformed by a wide Kommerell’s diverticulum (K), which is largely obscured by the trachea (*). B. A view showing the right aortic arch in a similar way as in Fig. 1A. Color-coded flow; however, branches of the aortic arch are not visible. C. A view showing the ascending aorta (AoAsc) in a sagittal plane. The trachea (*) is located just behind the aorta, slightly above the crossing with the right pulmonary artery (RPA), significantly deformed by a hyperechoic structure – the Kommerell’s diverticulum (K)

Fig. 9.

RAA – the ductus arteriosus from the descending aorta to the right pulmonary artery. A. Suprasternal view, frontal view; systole. The descending aorta (Desc) connects to the right pulmonary artery (RPA) via a wide vessel (*) with flow visible during both systole and diastole – a typical feature of the ductus arteriosus. B. The view and legends as in Fig 10 A. Diastole. Flow limited to the DA. C. A high right parasternal view, a section in the sagittal-like plane. Visible elements: the ascending aorta (AoAsc), the aortic arch (TrA), the right common carotid artery (RCCA), the right subclavian artery (RSA), the ductus arteriosus (*) arising from the descending aorta (Desc) and connecting to the right pulmonary artery (RPA)

Fig. 10.

RAA ALSA Kommerell’s diverticulum. A. A horizontal mediastinal section showing the right aortic arch (TrA) and its final branch – the left subclavian artery (LSA) located retrotracheally in its initial segment (*), which makes it very difficult to visualize its course. B. A slight parallel downward movement of the transducer allows for more precise visualization of the initial, wide segment of the left subclavian artery (Kommerell’s diverticulum – K) arising from the distal portion of the aortic arch (Desc; it runs approximately parallel, slightly above the right pulmonary artery – RPA). Furthermore, a transverse section of the pulmonary trunk is visible. C. The ductus arteriosus (DA) is still patent, representing a nearly smooth continuation of the Kommerell’s diverticulum (K). The ductal orifice to the pulmonary trunk (*) and a fragment of the right pulmonary artery (RPA) are visible. D. A parallel leftward movement of the transducer shows the further course of the LSA, which becomes significantly narrower after forming the ductus. A division into the distal LSA and the vertebral artery (VA) is seen. The left common carotid artery (LCCA) and the left subclavian vein (LSA) are visible anteriorly to the LSA

Fig. 11.

RAA with an aberrant subclavian artery, the ductus arteriosus arising directly from the descending aorta and connecting to the LPA. A. A high parasternal view showing the upper mediastinum in a transverse plane. Systole. Visible elements: the pulmonary artery trunk (PA), its left branch (LPA), distal transverse portion of the aortic arch (TrA). These vessels are coded blue. Red-coded flow in the ductus arteriosus (*) is seen between the descending aorta and the initial segment of the left pulmonary artery. The vascular structures form a complete vascular ring around the trachea and esophagus. B. View and legends as in Fig. 11 A. Diastole. Ductus arteriosus shunt into the initial segment of the left pulmonary artery and the pulmonary trunk is more visible during this phase of the cardiac cycle. C. A slight upward movement of the ultrasound beam visualizes both left-sided branches of the aortic arch: the left common carotid artery (LCCA) and the left subclavian artery (LSA), which arises separately from the distal part of the aortic arch

Fig. 12.

RAA – the left descending aorta. A series of images from scanning in the suprasternal, frontal view. A. Systole. Visible are both blue-coded pulmonary arteries, the right pulmonary artery is crossed by the ascending aorta (AoAsc), and the initial segment of the transverse part of the aortic arch, which gives rise to its first branch – the left common carotid artery (LCCA) directed to the left and upwards. A pathognomonic picture for right aortic arch. B. A slight posterior tilt of the ultrasound beam. The ascending aorta is no longer visible. Only the transverse part of the aortic arch with the left common carotid artery is still visible. C. Further posterior movement of the ultrasound beam reveals a wide transverse part of the aortic arch (TrA), which is obscured by the tracheal acoustic shadow (T). The aortic arch is directed leftward, while the descending aorta has a typical course, on the left side of the chest. The right common carotid artery (RCCA) arises from the arch most to the right. The left subclavian artery (LSA) is the final branch of the aortic arch; therefore it meets the criteria for an aberrant artery. It should be emphasized that it has no retroesphageal course, which is present in a typical form of right aortic arch with left aberrant subclavian artery as it arises from the aorta following the intersection with the esophagus and trachea; therefore, it causes no compression of any of these structures

Fig. 13.

RAA, complex forms. A. High parasternal view showing large arteries in a transverse section. On the right – the ascending aorta (AoAsc), on the left – the pulmonary artery (PA), with no wall between these vessels – an aortopulmonary window (W). The pulmonary artery gives rise to only one artery, i.e. the right pulmonary artery (RPA) dividing (after a long course) into the left pulmonary artery (LPA), which passes in an arch to the left, and typically coursing right pulmonary artery (RPA). A hyperechoic trachea is seen on the left from the site of left pulmonary artery origin – the arrangement of pulmonary arteries is typical for pulmonary sling. B. View and legends as in Fig. 13A, color-coded flow, systole. C. A view showing the upper mediastinum in a transverse section. The aortic arch (TrA) with its rightward convexity is shown; the first branch (the brachiocephalic trunk) runs leftwards (LTBC) – a pathognomonic picture for right aortic arch. Limited flow in the aortic arch coded in blue – the flow is directed downwards, moving away from the transducer. D. The flow in the aortic arch is reversed during diastole (coded in red), which is due to significant steal into the aortopulmonary window

Fig. 14.

A double aortic arch with preserved flow in both branches: A. A suprasternal projection showing the mediastinum in an intermediate plane between the frontal and transverse plane. A cross-section of the ascending aorta (AoAsc), the right pulmonary artery (RPA) crossing it from behind, a part of the left atrium (LA), and the descending aorta (Desc) located in the midline, behind its posterior wall, are visible. B. An upward tilt of the ultrasound beam, reaching a horizontal plane, visualizes the double aortic arch with a nearly symmetrical flow in both branches (RAA and LAA). Figures C–F are a series of images obtained in a high left parasternal view by gradually moving the ultrasound beam right-to-left in a sagittal plane: C. Structures located most to the right: the superior vena cava (SVC), the right pulmonary artery (RPA), a convex (to the right) part of the right-sided branch of the aortic arch (RAA). The ascending and descending aorta are not visible. D. A slight leftward tilt of the ultrasound beam shows the ascending aorta (AoAsc), the right branch of the aortic arch, and only the lower (pericardial) segment of the inferior vena cava. E. Further leftward tilt of the beam shows the pulmonary artery trunk (PA), the distal part of the left aortic arch and the segment passing medially, anteriorly to the spine (*), and the descending aorta behind pulmonary trunk bifurcation. F. Even further leftward movement of the ultrasound beam shows a longer segment of the left pulmonary artery (LPA) and the left convex part of the aortic arch (LAA). Again, the descending and ascending aorta are no longer visible

Fig. 15.

A double aortic arch with one of the segments being hypoplastic or atretic. The images were obtained by frontal-to-horizontal plane scanning: A. A suprasternal view, an intermediate plane between the frontal and horizontal plane. A cross-section of the ascending aorta (AoAsc), which is posteriorly crossed by the right pulmonary artery (RPA) is visible. The superior vena cava is seen on the right side of the aorta; the left atrium (LA) is located below and posteriorly to the RPA. The brachiocephalic vein is seen anteriorly and above the AoAsc. B. Horizontal plane: a wide vessel with a horizontal, leftward course is visible. C. An enlarged image for better visualization of the branch of the aortic arch from the previous image. A slight leftward and upward movement of the ultrasound beam shows the division of this vessel and its pronounced posterior curve. In particular, the more posteriorly located artery forms a pronounced pointed elbow. D. A slight rightward movement of the transducer shows a wide right aortic arch. It is not possible to show a vascular structure connecting the right aortic arch with elbow-like bended left subclavian artery

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