Abstract
Interruption of the aortic arch (IAA) is almost always associated with significant congenital heart disease resulting in clinical symptoms in the neonatal period. Diagnosis and description of additional cardiovascular malformations can be well established by echocardiography and colour Doppler. Interruption of the aortic arch is best visualized in the high right parasternal and in the suprasternal long-axis views. IAA type A is characterized by interruption distal to the left subclavian artery and type B by interruption distal to the left carotid artery. Care has to be taken not to misinterpret the “ductal arch” for the aortic arch. Colour Doppler is useful in this differentiation, showing bidirectional flow in the presence of a large ductus arteriosus. In addition, colour Doppler is essential in the detection and specification of possible subclavian artery anomalies. In the absence of ductal constriction, PW Doppler interrogation of the ductus arteriosus shows bidirectional flow with RL-shunting during systole and LR-shunting during diastole.
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22.1 Electronic Supplementary Material
Video 22.1
The high right parasternal long-axis view in a newborn shows interruption of the aortic arch distal to the left carotid and left subclavian artery representing interrupted aortic arch type A according to Celoria and Patton (same patient as in Videos 22.2 and 22.3). The right pulmonary artery is visualized in cross section below the ascending aorta and the innominate vein cranial to the ascending aorta (AVI 20325 kb)
Video 22.2
The ductal view confirms interruption of the aortic arch (same patient as in Videos 22.1 and 22.3). A large ductus arteriosus connects the pulmonary artery with the descending aorta. Care has to be taken not to confuse the “ductal arch” with the true aortic arch (AVI 6711 kb)
Video 22.3
Colour Doppler in the ductal view shows antegrade systolic and retrograde diastolic flow in the “ductal arch” (same patient as in Videos 22.1 and 22.3) (AVI 1107 kb)
Video 22.6
The ductal view in a patient with IAA type A reveals interruption of the aortic arch distal to the left subclavian artery, while the large ductus arteriosus connects the pulmonary artery with the descending aorta (AVI 5298 kb)
Video 22.7
This slightly oblique parasternal short-axis sweep starts in the plane of the pulmonary bifurcation. The pulmonary bifurcation is connected to the descending aorta by a large ductus arteriosus. Cranial tilt of the transducer reveals interruption of the aortic arch, while further cranial tilt displays the proximal aortic arch which ends distal to the left subclavian artery (AVI 39881 kb)
Video 22.8
The sweep in the high left parasternal sagittal view of a patient with IAA type B starts with a longitudinal view of the left common carotid artery, which represents the end of the proximal aortic arch. Lateral tilt of the transducer together with some clockwise rotation reveals interruption of the aortic arch and finally displays the main pulmonary artery and pulmonary bifurcation connected to the descending aorta by a large and unrestrictive ductus arteriosus (AVI 29128 kb)
Video 22.4
Posterior deviation of the infundibular septum, resulting in severe subaortic obstruction, is apparent in the parasternal long axis of a newborn with IAA type B and posterior malalignment VSD. Subaortic stenosis due to posterior deviation of the infundibular septum is a strong indicator for possible obstruction of the aortic arch (AVI 5786 kb)
Video 22.5
This parasternal short-axis sweep in a newborn with IAA type B starts at the level of the great arteries. The aorta is significantly smaller than the main pulmonary artery. Cranial tilt of the transducer displays the pulmonary bifurcation, and further tilt fails to reveal a transverse aortic arch. The ascending aorta divides into the innominate artery and the left common carotid artery, which represents the end of the aortic arch (AVI 31083 kb)
Video 22.9
The apical four-chamber view in a newborn with IAA type B shows enlargement of the right atrium and right ventricle. The large perimembranous VSD is not displayed in this view (AVI 12805 kb)
Video 22.10
The parasternal long-axis view of a newborn with IAA type B shows the large malalignment VSD with posterior deviation of the infundibular septum, resulting in severe subaortic obstruction. Note the discrepancy in size between the small aortic root and the large pulmonary artery (AVI 12692 kb)
Video 22.11
Colour Doppler in the parasternal long-axis view (same patient as in Video 22.10) fails to reveal acceleration of flow across the obstructed left ventricular outflow tract. This can be explained by the fact that only a limited amount of systemic outflow passes the left ventricular outflow to reach the brachiocephalic vessels (AVI 7220 kb)
Video 22.12
The parasternal short-axis view in a newborn with IAA type B displays the large, nonrestrictive perimembranous VSD (AVI 5485 kb)
Video 22.13
Significant LR-shunting across the nonrestrictive VSD is confirmed by colour Doppler (same patient as in Video 22.12) (AVI 1120 kb)
Video 22.14
Significant subaortic narrowing due to posterior deviation of the infundibular septum is shown in the apical five-chamber view of a newborn with IAA type B (AVI 6855 kb)
Video 22.15
Despite significant obstruction, colour Doppler shows no acceleration of flow across the left ventricular outflow (same patient as in Video 22.14) (AVI 1481 kb)
Video 22.16
Posterior deviation of the infundibular septum, the resulting posterior malalignment VSD and obstruction of the left ventricular outflow tract are nicely shown in the subcostal coronal view of a newborn with IAA type B (AVI 11881 kb)
Video 22.17
The parasternal short-axis view in this patient with IAA type B reveals bicuspid aortic valve (same patient as in Video 22.16) (AVI 15549 kb)
Video 22.18
Colour Doppler in the apical five-chamber view of another patient with IAA type B shows the large malalignment VSD, posterior deviation of the infundibular septum and left to right shunting across the VSD. Again there is absence of accelerated flow in the obstructed left ventricular outflow tract (AVI 6687 kb)
Video 22.19
Colour Doppler in the high left parasternal long-axis view of a newborn with IAA type B shows the large ductus arteriosus connecting the pulmonary artery with the descending aorta. Colour Doppler reveals bidirectional flow across the ductus arteriosus. Close to the aortic end of the ductus arteriosus, the left subclavian artery originates from the descending aorta confirming the diagnosis of IAA type B (AVI 1105 kb)
Video 22.20
Colour Doppler in a high right parasternal short-axis view with some clockwise rotation of the transducer in a patient with IAA type B and left aortic arch. The cranial vessel represents the right common carotid artery originating from the small ascending aorta. The right subclavian artery, which takes an aberrant origin from the descending aorta, is displayed with an inferior and parallel course to the right carotid artery (AVI 3417 kb)
Video 22.21
The longitudinal view of the right innominate artery in a newborn with IAA type B and left aortic arch shows absence of branching into common carotid and subclavian artery in the proximal part of this vessel (same patient as in Video 22.22) (AVI 2390 kb)
Video 22.22
Cranial angulation reveals the course of the innominate artery to the right side of the neck (same patient as in Video 22.22). Colour Doppler displays cervical origin of the right subclavian artery just below the bifurcation of right internal and external carotid artery (same patient as in Video 22.22) (AVI 48222 kb)
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Hofbeck, M., Deeg, KH., Rupprecht, T. (2017). Interruption of the Aortic Arch. In: Doppler Echocardiography in Infancy and Childhood. Springer, Cham. https://doi.org/10.1007/978-3-319-42919-9_22
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DOI: https://doi.org/10.1007/978-3-319-42919-9_22
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