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2013-09-22-18 Aortic arch anomalies © Bronshtein www.TheFetus.net


Aortic arch anomalies


Moshe Bronshtein
, MD*, Inna Naroditsky, MD*; Frantisek Grochal, MD**

* Haifa, Israel.
** Femicare, s.r.o., Center of prenatal ultrasonographic diagnostics, Martin, Slovak republic.

Case report

Formation of the great vessels of the heart and their branches is an early event during the embryogenesis. These structures originally come from paired aortic arches. The final aortic arch is created by parts left from the primitive aortic arches – its initial part comes from truncus arteriosus, ascendant part from the aortic sac, transverse arch from the left 4th aortic arch, and descending aorta comes from the left dorsal aorta. The right counterparts of the structures regress or are incorporated into other parts of the vascular system. Similarly ductus arteriosus normally comes from the left 6th aortic arch.

Image 1: The drawing shows embryological structures contributing to formation of the aortic arch (
its initial part comes from truncus arteriosus, ascendant part from the aortic sac, transverse arch comes from the left 4th aortic arch, and descending aorta comes from the left dorsal aorta).



In some cases the right parts of the paired aortic arches develops instead of the left ones, or they coexist together with their left-sided counterparts. This leads to the formation of some vascular anatomical variants as for example the right aortic arch, double aortic arch, right-sided ductus arteriosus, etc.

Some of these anatomical variants, their sonographic appearance or pathological correlations are presented in this article.

Image 2, video 1: The image 2 represents a drawing showing normal arrangement of the great vessels and some of their branches that should be seen during the transverse scanning through the thorax going from the four-chamber view of the heart up to the level of the three-vessel trachea view. The video 1 shows 4D imaging of the normal arrangement of the great vessels and ductus arteriosus (the ductus arteriosus in this case has a left-sided morphology, e.g. goes in straight continuation of the pulmonary artery towards the descending aorta).

PA - pulmonary artery; LDA - left ductus arteriosus; Ao - aorta; Left Ao - left-sided transverse aortic arch; DA - descending aorta; SVC - superior vena cava; Tr - trachea; LPA - left pulmonary artery; RPA - right pulmonary artery. 

 

Image 3, video 2: The image 3 represents a drawing showing arrangement of the great vessels in case of the right aortic arch seen during the transverse scanning through the thorax going from the four-chamber view of the heart up to the level of the three-vessel trachea view. The video 2 shows 4D imaging of arrangement of the great vessels and ductus arteriosus in case of the right aortic arch (the aortic arch goes on the right side of the trachea). Right-sided aortic arch and pulmonary artery make a vascular ring via the left sided ductus arteriosus (the ring goes behind the trachea).  The presence of the ring is usually asymptomatic in postnatal period. 

PA - pulmonary artery; LDA - left ductus arteriosus; Ao - aorta; RAA - right sided aortic arch; DA - descending aorta; SVC - superior vena cava; Tr - trachea; LPA - left pulmonary artery; RPA - right pulmonary artery. 


 

Images 4, 5, 6, and 7: The images 4-6 represent gray-scale and color Doppler images showing the transverse scans of the thorax at the level of the three-vessel trachea view in case of the right aortic arch. The image 7 represents a pathological correlate showing the dorsal view at the trachea entrapped by the vascular ring creating by the right-sided aorta and left ductus arteriosus.

PA - pulmonary artery; Left DA - left ductus arteriosus; Ao - aorta; DA - descending aorta; SVC - superior vena cava; Tr - trachea. 


 

 

Images 8, 9, 10, and video 3The image 8 represents a drawing showing arrangement of the structures in case of the right ductus arteriosus in combination with the right sided aortic arch. No vascular ring is present in this case.
The images 9, 10 and video 3 shows color Doppler scans showing the 
the right ductus arteriosus in combination with the right sided aortic arch. Note the angulation of the right ductus with the main pulmonary artery - the ductus directs to the right instead of going straight and dorsally towards the descending aorta. No vascular ring is present.

PA - pulmonary artery; RDA - right ductus arteriosus; Ao - aorta; RAA - right-sided aortic arch; DA - descending aorta; SVC - superior vena cava; Tr - trachea. 

 

 

Images 11, 12The image 11 represents a drawing showing arrangement of the structures in case of the right ductus arteriosus in combination with the normal left-sided aortic arch. No vascular ring is present in this case, but the ductus continues with an angulation from the pulmonary artery and directs to the right.
The images 12 shows gray-scale images of 
arrangement of the structures in case of the right ductus arteriosus in combination with the normal left-sided aortic arch. The ductus continues with angulation from the pulmonary artery and directs to the right. 

PA - pulmonary artery; RDA - right ductus arteriosus; Ao - aorta; Left Ao - left-sided aorta; DA - descending aorta; SVC - superior vena cava; Tr - trachea; RPA - right pulmonary artery; LPA - left pulmonary artery.

 

Images 13, 14, 15, 16: The images 11-12 represent gray-scale images of arrangement of the structures in a case of the right ductus arteriosus in combination with the normal left-sided aortic arch. For comparison the images 15-16 show normal variant of the left ductus arteriosus that is a direct continuation of the pulmonary artery towards the descending aorta.
 
MPA - main pulmonary artery; Ao - aorta; RDA - right ductus arteriosus; LDA left ductus arteriosus; RPA - right pulmonary artery.

 

 

Images 17, 18, 19, and video 4The image 17 represents a drawing showing arrangement of the structures in a case of the double aortic arch in combination with the right ductus. The double aortic arch creates a vascular ring entrapping the trachea and esophagus within the ring.

The images 18, 19, and video 4 show an example of the double aortic arch in combination with the right ductus (note his angulation with the main pulmonary artery). The ductus connects to the left-sided part of the double aortic arch. 
The double aortic arch creates a vascular ring entrapping the trachea and esophagus within the ring. Such finding has some clinical consequences (stridor, dysphagia) because the ring compresses the trachea and esophagus.

PA - pulmonary artery; RDA - right ductus arteriosus; Ao - aorta; Left Ao arch - left aortic arch of the doubled aortic arch; Right Ao arch - right aortic arch of the doubled aortic arch; DA - descending aorta; SVC - superior vena cava; Tr - trachea; RPA - right pulmonary artery; LPA - left pulmonary artery.

 

 

Images 20, 21, and video 5The image 20 represents a drawing showing arrangement of the structures in a case of the double aortic arch in combination with the left ductus arteriosus. The double aortic arch creates a vascular ring entrapping the trachea and esophagus within the ring. 

The image 20 and video 5 show an example of the double aortic arch in combination with the left ductus arteriosus that connects to the right-sided part of the double aortic arch. 
The double aortic arch creates a vascular ring entrapping the trachea and esophagus within the ring. Another vascular ring is made between the left ductus arteriosus and the right aortic arch of the doubled aortic arch. Such finding also leads to postnatal stridor and dysphagia because the ring of the doubled aortic arch compresses the trachea and esophagus.

PA - pulmonary artery; LDA - left ductus arteriosus; Ao - aorta; Left Ao arch - left aortic arch of the doubled aortic arch; Right Ao arch - right aortic arch of the doubled aortic arch; DA - descending aorta; SVC - superior vena cava; Tr - trachea; RPA - right pulmonary artery; LPA - left pulmonary artery.

 



Images 22, 23The image 22 shows postnatal X-ray of the esophagus compressed by the ring of the double aortic arch (arrow). The image 23 represents CT-angiogram of a case of the double aortic arch making a vascular ring entrapping the trachea and esophagus within the ring.

L-Ao - left aortic arch of the doubled aortic arch; R-Ao - right aortic arch of the doubled aortic arch.

 
 
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