Figure 1
Chest radiograph PA view
Absent left aortic contour,
presence of right aortic knob (red arrow), both hemidiaphragms are flat and low in position.
Right-sided aortic arch with aberrant left subclavian artery
SectionCardiovascular
Case TypeClinical Cases
AuthorsPradosh Kumar Sarangi1, Pratisruti Hui1, Sasmita Parida2
Connected authors
60 years, female
Clinical History
A 60-year-old female patient with known chronic obstructive pulmonary disease (COPD) presented with shortness of breath and dry cough for the past few days. She had no history of diabetes, hypertension or dysphagia.
Imaging Findings
Chest X-ray (Fig. 1) showed right-sided aortic knob with widening of the right mediastinum with hyperinflated lung and flattened diaphragms.
CT scanogram (Fig. 2) revealed right aortic arch with tracheal bowing to the left at the level of the right aortic arch.
CECT thorax (Fig. 3) confirmed right-sided aortic arch with right descending thoracic aorta. The first vessel originating from the right-sided arch is the left common carotid artery (CCA) [yellow arrow] which was followed by the right CCA [green arrow], right subclavian artery (RSA) [blue arrow] and left subclavian artery (LSA) [red arrow]. Here aberrant LSA originated from Kommerell’s diverticulum (focal dilatation of the arch) and coursed posterior to the oesophagus. Aortic arch was noted on the right side of the distal trachea (Fig. 3f)
Coronal 3D volume-rendering images in multiple views (Fig. 4) best describe the origin of four vessels from right-sided aortic arch with aberrant left subclavian artery originating from Kommerell’s diverticulum.
CT scanogram (Fig. 2) revealed right aortic arch with tracheal bowing to the left at the level of the right aortic arch.
CECT thorax (Fig. 3) confirmed right-sided aortic arch with right descending thoracic aorta. The first vessel originating from the right-sided arch is the left common carotid artery (CCA) [yellow arrow] which was followed by the right CCA [green arrow], right subclavian artery (RSA) [blue arrow] and left subclavian artery (LSA) [red arrow]. Here aberrant LSA originated from Kommerell’s diverticulum (focal dilatation of the arch) and coursed posterior to the oesophagus. Aortic arch was noted on the right side of the distal trachea (Fig. 3f)
Coronal 3D volume-rendering images in multiple views (Fig. 4) best describe the origin of four vessels from right-sided aortic arch with aberrant left subclavian artery originating from Kommerell’s diverticulum.
Discussion
A right aortic arch is present in about 0.1% of the population. It results from the persistence of the right fourth aortic arch and involution of the left. Here the arch crosses over the right main bronchus. It is classified into 3 types. [1, 2, 5]
1. Right aortic arch with mirror-image branching (59%): here the first to branch is the left innominate artery followed by the right common carotid and right subclavian arteries. It is usually associated with intracardiac defect, especially tetralogy of Fallot.
2. Right aortic arch with an aberrant left subclavian artery (39%)
3. Right aortic arch with an isolated left subclavian artery (0.8%): here the branches are the left common carotid, right common carotid and right subclavian arteries. The left subclavian artery is connected to the left pulmonary artery through a ductus arteriosus.
Chest anteroposterior radiographs and barium swallow studies may help in knowing the arch laterality from the pattern of indentation of the tracheal air column and oesophagus respectively. CT has emerged as the preferred mode of examination. [2]
In our case (type 2), the first trunk originating from the right-sided arch is the left common carotid artery (CCA) followed by the right CCA, right subclavian artery(RSA) and left subclavian artery (LSA). The aberrant LSA can arise from distal arch directly or more commonly from a Kommerell’s diverticulum (KD). [1-6] It is usually associated with conotruncal abnormalities such as tetralogy of Fallot or truncus arteriosus [3, 5]. KD is a remnant of the left fourth aortic arch between the left CCA and LSA and arises distal to the RSA coursing posteriorly to the oesophagus, supplying the LSA and usually is associated with left ductus forming a vascular ring. It can occur even in association with a left aortic arch with aberrant right subclavian artery (0.5–2.0%) [6]. A right aortic arch with an aberrant LSA and KD is the second most common cause of a symptomatic vascular ring, first being the double aortic arch. [2, 7, 8]
It is generally asymptomatic as the vascular ring is loose, but may present with dysphagia lusoria, atherosclerotic changes of the anomalous vessels, dissection and respiratory complaints. [1, 2]
Relief of symptoms like dysphagia or dyspnoea may be achieved by ligating and dividing the ligamentum arteriosum or by resecting a large diverticulum and anastomosing left subclavian artery with the left CCA. [2]
TEACHING POINT
Computed tomography is essential to study the vascular anatomy of right aortic arch anomalies in relation to the aerodigestive tract, and the presence of any vascular rings because these factors determine the surgical planning. [2, 5]
1. Right aortic arch with mirror-image branching (59%): here the first to branch is the left innominate artery followed by the right common carotid and right subclavian arteries. It is usually associated with intracardiac defect, especially tetralogy of Fallot.
2. Right aortic arch with an aberrant left subclavian artery (39%)
3. Right aortic arch with an isolated left subclavian artery (0.8%): here the branches are the left common carotid, right common carotid and right subclavian arteries. The left subclavian artery is connected to the left pulmonary artery through a ductus arteriosus.
Chest anteroposterior radiographs and barium swallow studies may help in knowing the arch laterality from the pattern of indentation of the tracheal air column and oesophagus respectively. CT has emerged as the preferred mode of examination. [2]
In our case (type 2), the first trunk originating from the right-sided arch is the left common carotid artery (CCA) followed by the right CCA, right subclavian artery(RSA) and left subclavian artery (LSA). The aberrant LSA can arise from distal arch directly or more commonly from a Kommerell’s diverticulum (KD). [1-6] It is usually associated with conotruncal abnormalities such as tetralogy of Fallot or truncus arteriosus [3, 5]. KD is a remnant of the left fourth aortic arch between the left CCA and LSA and arises distal to the RSA coursing posteriorly to the oesophagus, supplying the LSA and usually is associated with left ductus forming a vascular ring. It can occur even in association with a left aortic arch with aberrant right subclavian artery (0.5–2.0%) [6]. A right aortic arch with an aberrant LSA and KD is the second most common cause of a symptomatic vascular ring, first being the double aortic arch. [2, 7, 8]
It is generally asymptomatic as the vascular ring is loose, but may present with dysphagia lusoria, atherosclerotic changes of the anomalous vessels, dissection and respiratory complaints. [1, 2]
Relief of symptoms like dysphagia or dyspnoea may be achieved by ligating and dividing the ligamentum arteriosum or by resecting a large diverticulum and anastomosing left subclavian artery with the left CCA. [2]
TEACHING POINT
Computed tomography is essential to study the vascular anatomy of right aortic arch anomalies in relation to the aerodigestive tract, and the presence of any vascular rings because these factors determine the surgical planning. [2, 5]
Differential Diagnosis List
Right-sided aortic arch with aberrant left subclavian artery from Kommerell’s diverticulum
Double aortic arch with atretic left arch
Right paratracheal mass (most commonly lymphadenopathy)
Final Diagnosis
Right-sided aortic arch with aberrant left subclavian artery from Kommerell’s diverticulum
References
[1] Mubarak MY, Kamarul AT, Noordini MD (2011) Right-sided Aortic Arch with Aberrant Left Subclavian Artery from Kommerell’s Diverticulum. Iran J Radiol 8(2):103-6 (PMID: 23329925)
[2] M Etesami,Ashwath R, Kanne J, Gilkeson R.C (2014) Computed tomography in the evaluation of vascular rings and slings. Insights Imaging 5:507–521
[3] Backer CL, Mavroudis C (2000) Congenital Heart Surgery Nomenclature and Database Project: vascular rings, tracheal stenosis, pectus excavatum. Ann Thorac Surg 69(4 Suppl):S308-18 (PMID: 10798437)
[4] Knight L, Edwards JE (1974) Right aortic arch: types and associated cardiac anomalies. Circulation 50(5):1047-51 (PMID: 4430090)
[5] Ramos-Duran L, Nance JW Jr, Schoepf UJ, Henzler T, Apfaltrer P, Hlavacek AM (2012) Developmental aortic arch anomalies in infants and children assessed with CT angiography. AJR Am J Roentgenol 198(5):W466-74 (PMID: 22528928)
[6] Yang MH, Weng ZC, Weng YG, Chang HH (2009) A right-sided aortic arch with Kommerell's diverticulum of the aberrant left subclavian artery presenting with syncope. J Chin Med Assoc 72(5):275-7 (PMID: 19467953)
[7] Park SC (1981) Symposium on pediatric otolaryngology. Vascular abnormalities. Pediatr Clin North Am 28(4):949-55 (PMID: 7031580)
[8] Ekstrom G, Sandblom P (1951) Double aortic arch. Acta Chir Scand 102(3):183-202 (PMID: 14884907)
Case information
| URL: | https://www.eurorad.org/case/13605 |
| DOI: | 10.1594/EURORAD/CASE.13605 |
| ISSN: | 1563-4086 |
License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
Figure 3
CECT Thorax
Four vessels from the right aortic arch in the following order. Left CCA (yellow arrow), right CCA(green arrow), right SA(blue arrow), left SA (red arrow). Purple arrow-left subclavian vein, T-trachea, O-oesophagus
White arrow- right aortic arch, yellow arrow- left CCA, red arrow- aberrant left subclavian artery coursing posterior to oesophagus, S-superior vena cava, T-trachea, O-oesophagus
Aberrant left subclavian artery (red arrow) arising from Kommerell’s diverticulum (white arrow),
a focal dilatation of the arch.
Yellow arrow-left CCA,
S-superior vena cava
The descending aorta (D)continues inferiorly on the
right side of the vertebral column.
Thoracic aorta (white arrow), descending on the right side of the spine then turning to the left to enter the aortic hiatus at normal position.
Aortic arch (white arrow) on the right side of distal trachea.
Figure 4
3D volume-rendering images
Anterior view: Note the origin of 4 vessels from right-sided aortic arch in the following order.
1. left CCA
2. right CCA
3. right subclavian artery
4. left subclavian artery (last branch)
Anterior view, slightly rotated:
1. left CCA
2. right CCA
3. right subclavian artery
4. left subclavian artery (last branch)
5. Kommerell’s diverticulum
6. right-sided aortic arch
Posterior view:
Aberrant left subclavian artery (4) arising from Kommerell’s diverticulum(5), a focal dilatation of the arch.
6. right-sided aortic arch
7. descending aorta.
Lateral view:
1. left CCA
2. right CCA
3. right subclavian artery
4. left subclavian artery (last branch)
5. Kommerell’s diverticulum
6. right-sided aortic arch
7. descending aorta.
Lateral view, slight rotation:
1. left CCA
2. right CCA
3. right subclavian artery
4. left subclavian artery (last branch)
5. Kommerell’s diverticulum
6. right-sided aortic arch
7. descending aorta
Chest radiograph PA view
Absent left aortic contour,
presence of right aortic knob (red arrow), both hemidiaphragms are flat and low in position.
Department of radiodiagnosis, SCB medical college, cuttack, India
Department of radiodiagnosis, SCB medical college, cuttack, India
Scanogram
Right aortic arch (white arrow), distal tracheal deviation to the left (yellow arrow).
Dept. ofradiodiagnosis, SCB medical college, cuttack ,India
Dept. ofradiodiagnosis, SCB medical college, cuttack ,India
Lateral scanogram showing right aortic arch with right descending thoracic aorta.
Department of Radiodiagnosis, SCB medical college and hospital, Cuttack, India
Department of Radiodiagnosis, SCB medical college and hospital, Cuttack, India
CECT Thorax
Four vessels from the right aortic arch in the following order. Left CCA (yellow arrow), right CCA(green arrow), right SA(blue arrow), left SA (red arrow). Purple arrow-left subclavian vein, T-trachea, O-oesophagus
Department of Radiodiagnosis, SCB medical college and hospital, Cuttack, India
Department of Radiodiagnosis, SCB medical college and hospital, Cuttack, India
White arrow- right aortic arch, yellow arrow- left CCA, red arrow- aberrant left subclavian artery coursing posterior to oesophagus, S-superior vena cava, T-trachea, O-oesophagus
Department of Radiodiagnosis, SCB medical college and hospital, Cuttack, India
Department of Radiodiagnosis, SCB medical college and hospital, Cuttack, India
Aberrant left subclavian artery (red arrow) arising from Kommerell’s diverticulum (white arrow),
a focal dilatation of the arch.
Yellow arrow-left CCA,
S-superior vena cava
Department of Radiodiagnosis, SCB medical college and hospital, Cuttack, India
Department of Radiodiagnosis, SCB medical college and hospital, Cuttack, India
The descending aorta (D)continues inferiorly on the
right side of the vertebral column.
Department of Radiodiagnosis, SCB medical college and hospital, Cuttack, India
Department of Radiodiagnosis, SCB medical college and hospital, Cuttack, India
Thoracic aorta (white arrow), descending on the right side of the spine then turning to the left to enter the aortic hiatus at normal position.
Department of Radiodiagnosis, SCB medical college and hospital, Cuttack, India
Department of Radiodiagnosis, SCB medical college and hospital, Cuttack, India
Aortic arch (white arrow) on the right side of distal trachea.
Department of Radiodiagnosis, SCB medical college and hospital, Cuttack, India
Department of Radiodiagnosis, SCB medical college and hospital, Cuttack, India
3D volume-rendering images
Anterior view: Note the origin of 4 vessels from right-sided aortic arch in the following order.
1. left CCA
2. right CCA
3. right subclavian artery
4. left subclavian artery (last branch)
Department of radiodiagnosis,SCB medical college ,Cuttack,India
Department of radiodiagnosis,SCB medical college ,Cuttack,India
Anterior view, slightly rotated:
1. left CCA
2. right CCA
3. right subclavian artery
4. left subclavian artery (last branch)
5. Kommerell’s diverticulum
6. right-sided aortic arch
Department of radiodiagnosis,SCB medical college ,Cuttack,India
Department of radiodiagnosis,SCB medical college ,Cuttack,India
Posterior view:
Aberrant left subclavian artery (4) arising from Kommerell’s diverticulum(5), a focal dilatation of the arch.
6. right-sided aortic arch
7. descending aorta.
Department of radiodiagnosis,SCB medical college ,Cuttack,India
Department of radiodiagnosis,SCB medical college ,Cuttack,India
Lateral view:
1. left CCA
2. right CCA
3. right subclavian artery
4. left subclavian artery (last branch)
5. Kommerell’s diverticulum
6. right-sided aortic arch
7. descending aorta.
Department of radiodiagnosis,SCB medical college ,Cuttack,India
Department of radiodiagnosis,SCB medical college ,Cuttack,India
Lateral view, slight rotation:
1. left CCA
2. right CCA
3. right subclavian artery
4. left subclavian artery (last branch)
5. Kommerell’s diverticulum
6. right-sided aortic arch
7. descending aorta
Department of radiodiagnosis,SCB medical college ,Cuttack,India
Department of radiodiagnosis,SCB medical college ,Cuttack,India