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Left aortic arch with retroesophageal right subclavian artery

MedGen UID:
868867
•Concept ID:
C4023278
•
Anatomical Abnormality
HPO: HP:0011595

Definition

Aortic arch crosses the left mainstem bronchus. The first branch is the right carotid artery, the second branch is the left carotid artery, the third branch is the subclavian artery, the fourth branch is the right subclavian artery arising from the posteromedial aspect of the distal aortic arch and continuing posterior to the esophagus to the right hand side of the body. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLeft aortic arch with retroesophageal right subclavian artery

Conditions with this feature

VISS syndrome
MedGen UID:
1794165
•Concept ID:
C5561955
•
Disease or Syndrome
VISS syndrome is a generalized connective tissue disorder characterized by early-onset thoracic aortic aneurysm and other connective tissue findings, such as aneurysm and tortuosity of other arteries, joint hypermobility, skin laxity, and hernias, as well as craniofacial dysmorphic features, structural cardiac defects, skeletal anomalies, and motor developmental delay (Van Gucht et al., 2021). Immune dysregulation has been observed in some patients (Ziegler et al., 2021).
See: Condition Record
VISS syndrome

Recent clinical studies

Etiology

Morphological, embryological, and clinical implications of the bi-carotid trunk, aberrant right subclavian artery, and bilateral linguofacial trunk.
Chaudhary B, Kumari S
Surg Radiol Anat 2022 Nov;44(11):1461-1465. Epub 2022 Oct 23 doi: 10.1007/s00276-022-03036-0. PMID: 36273342
Anatomical characteristics of two cases of aberrant right subclavian artery.
Ito T, Itoh A, Kiyoshima D, Ikeda Y
Anat Sci Int 2022 Sep;97(4):423-427. Epub 2022 Mar 22 doi: 10.1007/s12565-022-00658-7. PMID: 35316514
Association of aberrant subclavian arteries with aortic pathology and proposed classification system.
Plotkin A, Ng B, Han SM, Weaver FA, Ham SW, Bowdish ME, Wilcox AG, Magee GA
J Vasc Surg 2020 Nov;72(5):1534-1543. Epub 2020 Mar 29 doi: 10.1016/j.jvs.2020.01.042. PMID: 32238310
Congenital abnormalities of the aortic arch: revisiting the 1964 Stewart classification.
Li S, Wen H, Liang M, Luo D, Qin Y, Liao Y, Ouyang S, Bi J, Tian X, Norwitz ER, Luo G
Cardiovasc Pathol 2019 Mar-Apr;39:38-50. Epub 2018 Dec 6 doi: 10.1016/j.carpath.2018.11.004. PMID: 30623879
Left aberrant subclavian artery. Systematic study in adult patients.
Tyczyński P, Michałowska I, Wolny R, Dobrowolski P, Łazarczyk H, Rybicka J, Hoffman P, Witkowski A
Int J Cardiol 2017 Aug 1;240:183-186. Epub 2017 Apr 25 doi: 10.1016/j.ijcard.2017.04.052. PMID: 28483478

Diagnosis

Ortner syndrome caused by aberrant right subclavian artery: A case report.
Chen YW, Chang SL, Wu NC, Shih YJ
Medicine (Baltimore) 2022 Dec 9;101(49):e32272. doi: 10.1097/MD.0000000000032272. PMID: 36626475Free PMC Article
Retroesophageal left brachiocephalic vein with an ectatic aberrant left subclavian artery: A rare double vascular loop.
Ojha V, Pandey NN, Verma M, Kumar S
J Card Surg 2020 Sep;35(9):2355-2356. Epub 2020 Jul 27 doi: 10.1111/jocs.14700. PMID: 32720395
Association of aberrant subclavian arteries with aortic pathology and proposed classification system.
Plotkin A, Ng B, Han SM, Weaver FA, Ham SW, Bowdish ME, Wilcox AG, Magee GA
J Vasc Surg 2020 Nov;72(5):1534-1543. Epub 2020 Mar 29 doi: 10.1016/j.jvs.2020.01.042. PMID: 32238310
Congenital abnormalities of the aortic arch: revisiting the 1964 Stewart classification.
Li S, Wen H, Liang M, Luo D, Qin Y, Liao Y, Ouyang S, Bi J, Tian X, Norwitz ER, Luo G
Cardiovasc Pathol 2019 Mar-Apr;39:38-50. Epub 2018 Dec 6 doi: 10.1016/j.carpath.2018.11.004. PMID: 30623879
MR imaging of subaortic and retroesophageal anomalous courses of the left brachiocephalic vein in the fetus.
Dong SZ, Zhu M
Sci Rep 2018 Oct 3;8(1):14781. doi: 10.1038/s41598-018-33033-6. PMID: 30283087Free PMC Article

Therapy

Safety and efficacy of a hybrid approach for repair of complicated aberrant subclavian arteries.
Gray SE, Scali ST, Feezor RJ, Beaver TM, Back MR, Upchurch GR Jr, Huber TS, Fatima J
J Vasc Surg 2020 Dec;72(6):1873-1882. Epub 2020 Jul 19 doi: 10.1016/j.jvs.2020.01.075. PMID: 32665182

Prognosis

AORTIC ARCH VARIANT AND COMMON CAROTID ARTERY AGENESIS.
Costa AL, Sousa I, Palma S, Leitão J
Port J Card Thorac Vasc Surg 2021 Nov 7;28(3):71-72. doi: 10.48729/pjctvs.205. PMID: 35333461
Retroesophageal right subclavian artery associated with a bicarotid trunk and an ectopic origin of vertebral arteries.
Buffoli B, Verzeletti V, Hirtler L, Rezzani R, Rodella LF
Surg Radiol Anat 2021 Sep;43(9):1491-1495. Epub 2021 Apr 15 doi: 10.1007/s00276-021-02746-1. PMID: 33856505Free PMC Article
Association of aberrant subclavian arteries with aortic pathology and proposed classification system.
Plotkin A, Ng B, Han SM, Weaver FA, Ham SW, Bowdish ME, Wilcox AG, Magee GA
J Vasc Surg 2020 Nov;72(5):1534-1543. Epub 2020 Mar 29 doi: 10.1016/j.jvs.2020.01.042. PMID: 32238310
Left aberrant subclavian artery. Systematic study in adult patients.
Tyczyński P, Michałowska I, Wolny R, Dobrowolski P, Łazarczyk H, Rybicka J, Hoffman P, Witkowski A
Int J Cardiol 2017 Aug 1;240:183-186. Epub 2017 Apr 25 doi: 10.1016/j.ijcard.2017.04.052. PMID: 28483478
Right-sided aortic arch.
Białowas J, Hreczecha J, Grzybiak M
Folia Morphol (Warsz) 2000;59(3):211-6. PMID: 10974792

Clinical prediction guides

Anatomical characteristics of two cases of aberrant right subclavian artery.
Ito T, Itoh A, Kiyoshima D, Ikeda Y
Anat Sci Int 2022 Sep;97(4):423-427. Epub 2022 Mar 22 doi: 10.1007/s12565-022-00658-7. PMID: 35316514
Kommerell Diverticulum: Right Aortic Arch with Anomalous Origin of Left Subclavian Artery and Duplicity of Right Vertebral Artery in a 16-Year-Old Girl.
Silveira JV, Junqueira FP, Silveira CG, Consolim-Colombo FM
Am J Case Rep 2019 Feb 21;20:228-232. doi: 10.12659/AJCR.912053. PMID: 30787266Free PMC Article
Congenital abnormalities of the aortic arch: revisiting the 1964 Stewart classification.
Li S, Wen H, Liang M, Luo D, Qin Y, Liao Y, Ouyang S, Bi J, Tian X, Norwitz ER, Luo G
Cardiovasc Pathol 2019 Mar-Apr;39:38-50. Epub 2018 Dec 6 doi: 10.1016/j.carpath.2018.11.004. PMID: 30623879
Anomalous origin of both vertebral arteries.
Gluncic V, Ivkic G, Marin D, Percac S
Clin Anat 1999;12(4):281-4. doi: 10.1002/(SICI)1098-2353(1999)12:4<281::AID-CA8>3.0.CO;2-6. PMID: 10398389
Retroesophageal right subclavian artery originating from the aortic arch distal and dorsal to the left subclavian artery.
Nakatani T, Tanaka S, Mizukami S, Okamoto K, Shiraishi Y, Nakamura T
Ann Anat 1996 Jun;178(3):269-71. doi: 10.1016/S0940-9602(96)80064-1. PMID: 8712376

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