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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

Assessment and management of heart failure in the systemic right ventricle.

Carazo M, Andrade L, Kim Y, Wilson W, Wu FM
Heart Fail Rev 2020 Jul;25(4):609-621. doi: 10.1007/s10741-020-09914-2. PMID: 31970551

Genetics and genetic testing in congenital heart disease.

Cowan JR, Ware SM
Clin Perinatol 2015 Jun;42(2):373-93, ix. Epub 2015 Apr 14 doi: 10.1016/j.clp.2015.02.009. PMID: 26042910

See all (220)

Recent clinical studies

Etiology

Anomalous Aortic Origin of a Coronary Artery.

Stephens EH, Jegatheeswaran A, Brothers JA, Ghobrial J, Karamlou T, Francois CJ, Krishnamurthy R, Dearani JA, Binsalamah Z, Molossi S, Mery CM
Ann Thorac Surg 2024 Jun;117(6):1074-1086. Epub 2024 Jan 30 doi: 10.1016/j.athoracsur.2024.01.016. PMID: 38302054

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

Native aorto-ostial coronary lesions on CT coronary angiogram.

Riley JYJ, Leong K, Mortensen KH, Ariff B, Gopalan D
Br J Radiol 2021 Jul 1;94(1123):20210211. Epub 2021 Jun 9 doi: 10.1259/bjr.20210211. PMID: 33989055 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

Nikaidoh Procedure: How I Teach It.

Morell VO
Ann Thorac Surg 2017 Nov;104(5):1446-1449. doi: 10.1016/j.athoracsur.2017.06.039. PMID: 29054209

See all (3100)

Diagnosis

Anomalous Aortic Origin of a Coronary Artery.

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

Dual-source computed tomography coronary artery imaging in children.

Secinaro A, Curione D, Mortensen KH, Santangelo TP, Ciancarella P, Napolitano C, Del Pasqua A, Taylor AM, Ciliberti P
Pediatr Radiol 2019 Dec;49(13):1823-1839. Epub 2019 Aug 22 doi: 10.1007/s00247-019-04494-2. PMID: 31440884

Ivemark Syndrome.

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

Scanning suprasternal echocardiography.

Goh TH, Venables AW
Br Heart J 1980 Feb;43(2):148-58. doi: 10.1136/hrt.43.2.148. PMID: 7362709 Free PMC Article

See all (3016)

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

Long-Term Outcomes After Atrial Switch Operation for Transposition of the Great Arteries.

Broberg CS, van Dissel A, Minnier J, Aboulhosn J, Kauling RM, Ginde S, Krieger EV, Rodriguez F 3rd, Gupta T, Shah S, John AS, Cotts T, Kay WA, Kuo M, Dwight C, Woods P, Nicolarsen J, Sarubbi B, Fusco F, Antonova P, Fernandes S, Grewal J, Cramer J, Khairy P, Gallego P, O'Donnell C, Hannah J, Dellborg M, Rodriguez-Monserrate CP, Muhll IV, Pylypchuk S, Magalski A, Han F, Lubert AM, Kay J, Yeung E, Roos-Hesselink J, Baker D, Celermajer DS, Burchill LJ, Wilson WM, Wong J, Kutty S, Opotowsky AR
J Am Coll Cardiol 2022 Sep 6;80(10):951-963. doi: 10.1016/j.jacc.2022.06.020. PMID: 36049802

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

Cor triatriatum sinister.

Kadner A, Meszaros K, Mueller C, Schoenhoff F, Hutter D, Carrel T
Multimed Man Cardiothorac Surg 2014;2014 Epub 2014 May 30 doi: 10.1093/mmcts/mmu005. PMID: 24878580

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 (1324)

Prognosis

Growth of the Neo-Aortic Root and Prognosis of Transposition of the Great Arteries.

Sengupta A, Carreon CK, Gauvreau K, Lee JM, Sanders SP, Colan SD, Del Nido PJ, Mayer JE Jr, Nathan M
J Am Coll Cardiol 2024 Jan 30;83(4):516-527. Epub 2023 Nov 6 doi: 10.1016/j.jacc.2023.10.023. PMID: 37939977

Toward More Granular Guidelines in AAOCA: Associating Anatomical Details With Specific Surgical Strategies.

Jegatheeswaran A, DeCampli WM
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2023;26:63-74. Epub 2022 Dec 28 doi: 10.1053/j.pcsu.2022.12.007. PMID: 36842800

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

STRUCTURAL HEART ANOMALIES (REVIEW).

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

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 (2266)

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

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

Normal cerebral arterial development and variations.

Kathuria S, Gregg L, Chen J, Gandhi D
Semin Ultrasound CT MR 2011 Jun;32(3):242-51. doi: 10.1053/j.sult.2011.02.002. PMID: 21596279

See all (1370)

Recent systematic reviews

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

Systematic review and meta-analysis of preoperative interventions to support the maturation of arteriovenous fistulae in patients with advanced kidney disease.

Ramanarayanan S, Sharma S, Swift O, Laws KR, Umar H, Farrington K
Nephrol Dial Transplant 2023 Sep 29;38(10):2330-2339. doi: 10.1093/ndt/gfad040. PMID: 36805738 Free PMC Article

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

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

See all (29)

MedGen

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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

Recent activity