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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).

Recent clinical studies

Etiology

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
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
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
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
Ismat FA, Weinberg PM, Rychik J, Karl TR, Fogel MA
Congenit Heart Dis 2006 Sep;1(5):217-23. doi: 10.1111/j.1747-0803.2006.00038.x. PMID: 17330153Free PMC Article

Diagnosis

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
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
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
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
Dong SZ, Zhu M
Sci Rep 2018 Oct 3;8(1):14781. doi: 10.1038/s41598-018-33033-6. PMID: 30283087Free PMC Article

Therapy

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

Martín-González I, Díez-Solórzano ML, Blasco-Serra A, Valverde-Navarro AA, Colás-López A, Linuesa-Mendoza M, Cortés-Aucejo JJ, Ekuaga-Eyang MM, Jara-Jaquez A, Cortés-Martínez MC
Surg Radiol Anat 2022 Apr;44(4):531-534. Epub 2022 Mar 9 doi: 10.1007/s00276-022-02903-0. PMID: 35266028
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
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
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
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

Clinical prediction guides

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
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
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
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
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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