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Great Vessels Abnormality

MedGen UID:: 476191
•Concept ID:: C3274558
•: Congenital Abnormality

Synonym:

Great vessel abnormalities

Definition

A congenital vascular abnormality that affects the great vessels and/or aortic arch. Representative examples include double aortic arch, aortic coarctation, and absence of a pulmonary artery. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVGreat Vessels Abnormality

Phenotypic abnormality
- Abnormality of prenatal development or birth
  - Fetal anomaly
    - Congenital Systemic Disorder
      - Congenital anomaly of cardiovascular system
        Great Vessels Abnormality
        Aortic arch interruption
        Interrupted aortic arch type A
        Interrupted aortic arch type B
        Interrupted aortic arch type C
        Right aortic arch
        Right aortic arch with left descending aorta and left ductus arteriosus
        Right aortic arch with mirror image branching
        Right aortic arch with retroesophageal diverticulum of Kommerell
        Right aortic arch with retroesophageal left subclavian artery
        Transposition of the great arteries
        Congenital heart defects, multiple types, 6
        Congenitally corrected transposition of the great arteries
        Congenitally corrected transposition of the great arteries with ventricular septal defect
        D - transposition of the great vessels
        Dextro-looped transposition of the great arteries
        Congenitally uncorrected transposition of the great arteries with cardiac malformation
        Congenitally uncorrected transposition of the great arteries with coarctation
        Isolated congenitally uncorrected transposition of the great arteries
        Dextro-Transposition of the Great Vessels with Intact Ventricular Septum
        Dextro-Transposition of the Great Vessels with Ventricular Septal Defect
        Dextrotransposition of the great arteries
        Double outlet right ventricle
        Double outlet right ventricle with atrioventricular septal defect, pulmonary stenosis, heterotaxy
        Double outlet right ventricle with doubly committed ventricular septal defect and pulmonary stenosis
        Double outlet right ventricle with doubly committed ventricular septal defect without pulmonary stenosis
        Double outlet right ventricle with non-committed subpulmonary ventricular septal defect
        Double outlet right ventricle with non-committed ventricular septal defect and pulmonary stenosis
        Double outlet right ventricle with non-committed ventricular septal defect without pulmonary stenosis
        Double outlet right ventricle with subaortic or doubly committed ventricular septal defect
        Double outlet right ventricle with subaortic or doubly committed ventricular septal defect with pulmonary stenosis
        Double outlet right ventricle with subaortic ventricular septal defect and pulmonary stenosis
        Double outlet right ventricle with subaortic ventricular septal defect without pulmonary stenosis
        Double outlet right ventricle with subpulmonary ventricular septal defect
        Double outlet right ventricle with subpulmonary ventricular septal defect and pulmonary stenosis
        Double outlet right ventricle with subpulmonary ventricular septal defect without pulmonary stenosis
        Heterotaxy, visceral, 2, autosomal
        Levo-Transposition of the Great Arteries
        Levotransposition of the great arteries
        Transposition of the great arteries, dextro-looped
        Vascular ring

Professional guidelines

PubMed

Human Laterality Disorders: Pathogenesis, Clinical Manifestations, Diagnosis, and Management.

Soofi M, Alpert MA, Barbadora J, Mukerji B, Mukerji V
Am J Med Sci 2021 Sep;362(3):233-242. Epub 2021 May 28 doi: 10.1016/j.amjms.2021.05.020. PMID: 34052215

Tracheobronchomalacia, Tracheobronchial Compression, and Tracheobronchial Malformations: Diagnostic and Treatment Strategies.

Kamran A, Baird CW, Jennings RW
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2020;23:53-61. doi: 10.1053/j.pcsu.2020.02.006. PMID: 32354548

Diagnosis and management of life-threatening cardiac malformations in the newborn.

Mellander M
Semin Fetal Neonatal Med 2013 Oct;18(5):302-10. Epub 2013 May 18 doi: 10.1016/j.siny.2013.04.007. PMID: 23688937

See all (218)

Recent clinical studies

Etiology

Transpositions of the great arteries versus aortic dextropositions. A review of some embryogenetic and morphological aspects.

Restivo A, di Gioia C, Marino B, Putotto C
Anat Rec (Hoboken) 2023 Mar;306(3):502-514. Epub 2022 Dec 9 doi: 10.1002/ar.25129. PMID: 36426596

CT of transposition of the great arteries in adults.

Panayiotou A, Thorne S, Hudsmith LE, Holloway B
Clin Radiol 2022 Apr;77(4):e261-e268. Epub 2021 Dec 31 doi: 10.1016/j.crad.2021.12.006. PMID: 34980460

Diagnosis of major heart defects by routine first-trimester ultrasound examination: association with increased nuchal translucency, tricuspid regurgitation and abnormal flow in ductus venosus.

Minnella GP, Crupano FM, Syngelaki A, Zidere V, Akolekar R, Nicolaides KH
Ultrasound Obstet Gynecol 2020 May;55(5):637-644. doi: 10.1002/uog.21956. PMID: 31875326

Transposition of the great arteries.

Squarcia U, Macchi C
Curr Opin Pediatr 2011 Oct;23(5):518-22. doi: 10.1097/MOP.0b013e32834aa551. PMID: 21849902

Angiomegaly.

D'Andrea V, Malinovsky L, Cavallotti C, Benedetti Valentini F, Malinovska V, Bartolo M, Todini AR, Biancari F, Di Matteo FM, De Antoni E
J Cardiovasc Surg (Torino) 1997 Oct;38(5):447-55. PMID: 9358801

See all (3056)

Diagnosis

Diagnosis of major heart defects by routine first-trimester ultrasound examination: association with increased nuchal translucency, tricuspid regurgitation and abnormal flow in ductus venosus.

Minnella GP, Crupano FM, Syngelaki A, Zidere V, Akolekar R, Nicolaides KH
Ultrasound Obstet Gynecol 2020 May;55(5):637-644. doi: 10.1002/uog.21956. PMID: 31875326

Contemporary management and outcomes in congenitally corrected transposition of the great arteries.

Kutty S, Danford DA, Diller GP, Tutarel O
Heart 2018 Jul;104(14):1148-1155. Epub 2018 Jan 11 doi: 10.1136/heartjnl-2016-311032. PMID: 29326110

Ivemark Syndrome.

Masiwal P, Chenthil KS, Priyadarsini B, Gnanaprakasam J, Srihari I
J Assoc Physicians India 2016 May;64(5):73-75. PMID: 27735157

Transposition of the great arteries.

Squarcia U, Macchi C
Curr Opin Pediatr 2011 Oct;23(5):518-22. doi: 10.1097/MOP.0b013e32834aa551. PMID: 21849902

Angiomegaly.

See all (2978)

Therapy

Congenital heart defects caused by FOXJ1.

Padua MB, Helm BM, Wells JR, Smith AM, Bellchambers HM, Sridhar A, Ware SM
Hum Mol Genet 2023 Jul 4;32(14):2335-2346. doi: 10.1093/hmg/ddad065. PMID: 37158461 Free PMC Article

Predictors of mortality after atrial correction of transposition of the great arteries.

Antonová P, Rohn V, Chaloupecky V, Simkova I, Kaldararova M, Zeman J, Popelova J, Havova M, Janousek J
Heart 2022 Nov 10;108(23):1881-1886. doi: 10.1136/heartjnl-2021-320035. PMID: 35851319

Non-Recurrent Laryngeal Nerve and Concurrent Vascular Variants: A Review.

Bakalinis E, Makris I, Demesticha T, Tsakotos G, Skandalakis P, Filippou D
Acta Med Acad 2018 Nov;47(2):186-192. doi: 10.5644/ama2006-124.230. PMID: 30585070

Broad targeting of angiogenesis for cancer prevention and therapy.

Wang Z, Dabrosin C, Yin X, Fuster MM, Arreola A, Rathmell WK, Generali D, Nagaraju GP, El-Rayes B, Ribatti D, Chen YC, Honoki K, Fujii H, Georgakilas AG, Nowsheen S, Amedei A, Niccolai E, Amin A, Ashraf SS, Helferich B, Yang X, Guha G, Bhakta D, Ciriolo MR, Aquilano K, Chen S, Halicka D, Mohammed SI, Azmi AS, Bilsland A, Keith WN, Jensen LD
Semin Cancer Biol 2015 Dec;35 Suppl(Suppl):S224-S243. Epub 2015 Jan 16 doi: 10.1016/j.semcancer.2015.01.001. PMID: 25600295 Free PMC Article

Isotretinoin and pregnancy.

Stern RS, Rosa F, Baum C
J Am Acad Dermatol 1984 May;10(5 Pt 1):851-4. doi: 10.1016/s0190-9622(84)80142-5. PMID: 6233342

See all (1307)

Prognosis

Diagnosis of major heart defects by routine first-trimester ultrasound examination: association with increased nuchal translucency, tricuspid regurgitation and abnormal flow in ductus venosus.

Minnella GP, Crupano FM, Syngelaki A, Zidere V, Akolekar R, Nicolaides KH
Ultrasound Obstet Gynecol 2020 May;55(5):637-644. doi: 10.1002/uog.21956. PMID: 31875326

Computed Tomography Angiography and Magnetic Resonance Angiography of Congenital Anomalies of Pulmonary Veins.

Abdel Razek AAK, Al-Marsafawy H, Elmansy M, El-Latif MA, Sobh D
J Comput Assist Tomogr 2019 May/Jun;43(3):399-405. doi: 10.1097/RCT.0000000000000857. PMID: 31082945

STRUCTURAL HEART ANOMALIES (REVIEW).

Osovska N, Kuzminova N, Ovcharuk M, Serhiychuk O
Georgian Med News 2016 Jun;(255):66-77. PMID: 27441539

Great cardiac vein variations.

Bales GS
Clin Anat 2004 Jul;17(5):436-43. doi: 10.1002/ca.10248. PMID: 15176044

Arterial switch.

Planche C, Lacour-Gayet F, Serraf A
Pediatr Cardiol 1998 Jul-Aug;19(4):297-307. doi: 10.1007/s002469900313. PMID: 9636253

See all (2242)

Clinical prediction guides

Four-dimensional flow cardiovascular magnetic resonance in tetralogy of Fallot: a systematic review.

Elsayed A, Gilbert K, Scadeng M, Cowan BR, Pushparajah K, Young AA
J Cardiovasc Magn Reson 2021 May 20;23(1):59. doi: 10.1186/s12968-021-00745-0. PMID: 34011372 Free PMC Article

Diagnosis of major heart defects by routine first-trimester ultrasound examination: association with increased nuchal translucency, tricuspid regurgitation and abnormal flow in ductus venosus.

Minnella GP, Crupano FM, Syngelaki A, Zidere V, Akolekar R, Nicolaides KH
Ultrasound Obstet Gynecol 2020 May;55(5):637-644. doi: 10.1002/uog.21956. PMID: 31875326

Echocardiography in Congenital Heart Disease.

Mcleod G, Shum K, Gupta T, Chakravorty S, Kachur S, Bienvenu L, White M, Shah SB
Prog Cardiovasc Dis 2018 Nov-Dec;61(5-6):468-475. Epub 2018 Nov 13 doi: 10.1016/j.pcad.2018.11.004. PMID: 30445162

Unravelling cardiovascular disease using four dimensional flow cardiovascular magnetic resonance.

Kamphuis VP, Westenberg JJM, van der Palen RLF, Blom NA, de Roos A, van der Geest R, Elbaz MSM, Roest AAW
Int J Cardiovasc Imaging 2017 Jul;33(7):1069-1081. Epub 2016 Nov 25 doi: 10.1007/s10554-016-1031-9. PMID: 27888419 Free PMC Article

Quantification of systemic right ventricle by echocardiography.

Iriart X, Roubertie F, Jalal Z, Thambo JB
Arch Cardiovasc Dis 2016 Feb;109(2):120-7. Epub 2016 Feb 2 doi: 10.1016/j.acvd.2015.11.008. PMID: 26850171

See all (1347)

Recent systematic reviews

Long-term outcome of patients with transposition of the great arteries and a systemic right ventricle: A systematic review and meta-analysis.

Ansari Ramandi MM, Yarmohammadi H, Gareb B, Voors AA, van Melle JP
Int J Cardiol 2023 Oct 15;389:131159. Epub 2023 Jul 9 doi: 10.1016/j.ijcard.2023.131159. PMID: 37433408

Clavicular bone defects managed with free vascularised fibular grafting: evidence to date.

Wakefield SM, Giannoudis VP, Giannoudis PV
Eur J Orthop Surg Traumatol 2023 Dec;33(8):3307-3318. Epub 2023 Jun 8 doi: 10.1007/s00590-023-03598-8. PMID: 37289244 Free PMC Article

The association between maternal air pollution exposure and the incidence of congenital heart diseases in children: A systematic review and meta-analysis.

Wan X, Wei S, Wang Y, Jiang J, Lian X, Zou Z, Li J
Sci Total Environ 2023 Sep 20;892:164431. Epub 2023 Jun 1 doi: 10.1016/j.scitotenv.2023.164431. PMID: 37268121

Outcome of Pregnancy in Women With D-Transposition of the Great Arteries: A Systematic Review.

Pizula J, Devera J, Ng TMH, Yeung SL, Thangathurai J, Herrick N, Chatfield AJ, Mehra A, Elkayam U
J Am Heart Assoc 2022 Dec 6;11(23):e026862. Epub 2022 Nov 29 doi: 10.1161/JAHA.122.026862. PMID: 36444833 Free PMC Article

Arterial Stiffness in Congenital Heart Disease.

Sandhu K, Pepe S, Smolich JJ, Cheung MMH, Mynard JP
Heart Lung Circ 2021 Nov;30(11):1602-1612. Epub 2021 Aug 20 doi: 10.1016/j.hlc.2021.07.018. PMID: 34420886

See all (30)

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Great Vessels Abnormality

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

Supplemental Content

Table of contents

Practice guidelines

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