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Congenitally corrected transposition of the great arteries with ventricular septal defect

MedGen UID:: 868861
•Concept ID:: C4023272
•: Congenital Abnormality

Synonyms:	ccTGA; CCTGA

HPO:	HP:0011605

Definition

A congenitally corrected transposition of the great arteries with a ventricular septal defect [from HPO]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVCongenitally corrected transposition of the great arteries with ventricular septal defect

Abnormal cardiovascular system morphology
- Abnormal vascular morphology
  - Abnormal blood vessel morphology
    - Abnormal morphology of the great vessels
      - Congenital malformation of the great arteries
        Congenitally corrected transposition of the great arteries
        Congenitally corrected transposition of the great arteries with ventricular septal defect

Conditions with this feature

Heterotaxy, visceral, 8, autosomal

MedGen UID:: 934635
•Concept ID:: C4310668
•: Disease or Syndrome

Autosomal visceral heterotaxy-8 (HTX8) is an autosomal recessive developmental disorder characterized by visceral situs inversus associated with complex congenital heart malformations caused by defects in the normal left-right asymmetric positioning of internal organs (summary by Vetrini et al., 2016). For a discussion of the genetic heterogeneity of visceral heterotaxy, see HTX1 (306955).

See: Condition Record

Heterotaxy, visceral, 8, autosomal

Professional guidelines

PubMed

Anatomic Repair of Congenitally Corrected Transposition: Reappraisal of Eligibility Criteria.

Weixler VHM, Kramer P, Murin P, Romanchenko O, Cho MY, Ovroutski S, Hübler M, Berger F, Photiadis J
Pediatr Cardiol 2022 Aug;43(6):1214-1222. Epub 2022 Feb 11 doi: 10.1007/s00246-022-02841-z. PMID: 35149898 Free PMC Article

Postnatal Outcome Following Prenatal Diagnosis of Discordant Atrioventricular and Ventriculoarterial Connections.

Day TG, Woodgate T, Knee O, Zidere V, Vigneswaran T, Charakida M, Miller O, Sharland G, Simpson J
Pediatr Cardiol 2019 Oct;40(7):1509-1515. Epub 2019 Jul 25 doi: 10.1007/s00246-019-02176-2. PMID: 31342118

Management of the Adult Patient With Congenitally Corrected Transposition: Challenges and Uncertainties.

Connolly HM, Miranda WR, Egbe AC, Warnes CA
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2019;22:61-65. doi: 10.1053/j.pcsu.2019.02.006. PMID: 31027566

See all (4)

Recent clinical studies

Etiology

Status of adult outpatients with congenital heart disease in Japan: The Japanese Network of Cardiovascular Departments for Adult Congenital Heart Disease Registry.

Yao A, Inuzuka R, Mizuno A, Iwano H, Tatebe S, Tsukamoto Y, Sakamoto I, Watanabe H, Fukuda N, Takechi F, Adachi S, Akazawa Y, Kuwahara K, Dohi K, Ishizu T, Miyake M, Koitabashi N, Hasegawa-Tamba S, Sato S, Fujii T, Ehara E, Minamino T, Yamada H, Yamashita E, Kawamatsu N, Masuda K, Soma K, Shiraishi I, Nagai R, Niwa K; JNCVD-ACHD investigators
J Cardiol 2022 Dec;80(6):525-531. Epub 2022 Aug 19 doi: 10.1016/j.jjcc.2022.07.019. PMID: 35995687

Senning With Aortic Translocation and Anatomic Repair for Congenitally Corrected Transposition.

Tam VKH, Erez E, Roten L, Muyskens S, Sebastian V, Tsao C, Nikaidoh H
Ann Thorac Surg 2021 May;111(5):1607-1612. Epub 2020 Oct 10 doi: 10.1016/j.athoracsur.2020.07.056. PMID: 33045205

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

Transposition of the great arteries.

Warnes CA
Circulation 2006 Dec 12;114(24):2699-709. doi: 10.1161/CIRCULATIONAHA.105.592352. PMID: 17159076

Technical aspects of the Rastelli and atrial switch procedure for congenitally corrected transposition of the great arteries with ventricular septal defect and pulmonary stenosis or atresia: results of therapy.

Brawn WJ, Barron DJ
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2003;6:4-8. doi: 10.1053/pcsu.2003.50014. PMID: 12740766

See all (84)

Diagnosis

Management of the Adult Patient With Congenitally Corrected Transposition: Challenges and Uncertainties.

Connolly HM, Miranda WR, Egbe AC, Warnes CA
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2019;22:61-65. doi: 10.1053/j.pcsu.2019.02.006. PMID: 31027566

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

Transposition of the great arteries.

Warnes CA
Circulation 2006 Dec 12;114(24):2699-709. doi: 10.1161/CIRCULATIONAHA.105.592352. PMID: 17159076

Physiologic versus anatomic repair of congenitally corrected transposition of the great arteries.

Mavroudis C, Backer CL
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2003;6:16-26. doi: 10.1053/pcsu.2003.50015. PMID: 12740768

Brawn WJ, Barron DJ
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2003;6:4-8. doi: 10.1053/pcsu.2003.50014. PMID: 12740766

See all (70)

Therapy

Contemporary outcomes of the double switch operation for congenitally corrected transposition of the great arteries.

Marathe SP, Chávez M, Schulz A, Sleeper LA, Marx GR, Emani SM, Del Nido PJ, Baird CW
J Thorac Cardiovasc Surg 2022 Dec;164(6):1980-1990.e7. Epub 2022 Mar 11 doi: 10.1016/j.jtcvs.2022.01.049. PMID: 35688715

Iatrogenic ventricular septal defect during right ventricular assist device insertion in congenitally corrected transposition of the great arteries.

Deng MX, Jeewa A, Honjo O
J Card Surg 2022 Apr;37(4):1080-1082. Epub 2022 Jan 27 doi: 10.1111/jocs.16277. PMID: 35083800

Fate of the Left Ventricular Outflow Tract After Rastelli With Selective Infundibular Muscle Resection.

Barron DJ, Mehsood DK, Kutty RS, Stickley J, Botha P, Khan NE, Jones TJ, Brawn WJ
Ann Thorac Surg 2019 Apr;107(4):1226-1231. Epub 2018 Dec 6 doi: 10.1016/j.athoracsur.2018.10.069. PMID: 30529669

Anatomy of the ventricular septal defect in congenital heart defects: a random association?

Mostefa-Kara M, Houyel L, Bonnet D
Orphanet J Rare Dis 2018 Jul 18;13(1):118. doi: 10.1186/s13023-018-0861-z. PMID: 30021599 Free PMC Article

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

See all (29)

Prognosis

Contemporary outcomes of the double switch operation for congenitally corrected transposition of the great arteries.

Double Switch in Congenitally Corrected Transposition of the Great Arteries With Incomplete Atrioventricular Septal Defect, Ebsteinoid Tricuspid Valve, Ventricular Outflow Tract Obstructions, and Multiple VSDs.

Najm HK, Tucker D, Patel V, Karamlou T, Oh NA
World J Pediatr Congenit Heart Surg 2022 Jul;13(4):527-529. Epub 2022 Mar 11 doi: 10.1177/21501351221085544. PMID: 35275017

Transposition of the great arteries.

Warnes CA
Circulation 2006 Dec 12;114(24):2699-709. doi: 10.1161/CIRCULATIONAHA.105.592352. PMID: 17159076

Physiologic versus anatomic repair of congenitally corrected transposition of the great arteries.

Mavroudis C, Backer CL
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2003;6:16-26. doi: 10.1053/pcsu.2003.50015. PMID: 12740768

Brawn WJ, Barron DJ
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2003;6:4-8. doi: 10.1053/pcsu.2003.50014. PMID: 12740766

See all (55)

Clinical prediction guides

Anatomic Repair of Congenitally Corrected Transposition: Reappraisal of Eligibility Criteria.

Incidence and clinical characteristics of sudden cardiac death in adult congenital heart disease.

Moore B, Yu C, Kotchetkova I, Cordina R, Celermajer DS
Int J Cardiol 2018 Mar 1;254:101-106. Epub 2017 Dec 5 doi: 10.1016/j.ijcard.2017.11.117. PMID: 29224925

Single-Center Experience With the Senning Procedure in the Current Era.

Ferro G, Murthy R, Sebastian VA, Guleserian KJ, Forbess JM
Semin Thorac Cardiovasc Surg 2016 Summer;28(2):514-520. Epub 2016 Jan 8 doi: 10.1053/j.semtcvs.2015.12.013. PMID: 28043470

Midterm results of anatomic repair in a subgroup of corrected transposition.

Tocharoenchok T, Sriyoschati S, Tongcharoen P, Tantiwongkosri K, Subtaweesin T
Asian Cardiovasc Thorac Ann 2016 Jun;24(5):428-34. Epub 2016 Apr 19 doi: 10.1177/0218492316645749. PMID: 27095702

Anatomic repair for corrected transposition with left ventricular outflow tract obstruction.

Hoashi T, Kagisaki K, Miyazaki A, Kurosaki K, Shiraishi I, Yagihara T, Ichikawa H
Ann Thorac Surg 2013 Aug;96(2):611-20. Epub 2013 Jun 4 doi: 10.1016/j.athoracsur.2013.03.095. PMID: 23743063

See all (29)

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Congenitally corrected transposition of the great arteries with ventricular septal defect

Definition

Term Hierarchy

Conditions with this feature

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Supplemental Content

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