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Dextro-Transposition of the Great Vessels with Ventricular Septal Defect

MedGen UID:
768660
•Concept ID:
C3640020
•
Disease or Syndrome
Synonym: D-Transposition of the Great Vessels with Ventricular Septal Defect

Definition

A congenital heart defect in which there is an abnormal arrangement of any of the primary blood vessels of the heart in conjunction with a pathologic opening between the cardiac ventricles. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDextro-Transposition of the Great Vessels with Ventricular Septal Defect

Professional guidelines

PubMed

Population trends in prenatal detection of transposition of great arteries: impact of obstetric screening ultrasound guidelines.
Ravi P, Mills L, Fruitman D, Savard W, Colen T, Khoo N, Serrano-Lomelin J, Hornberger LK
Ultrasound Obstet Gynecol 2018 May;51(5):659-664. doi: 10.1002/uog.17496. PMID: 28436133
Transposition of the great arteries. Results of treatment with early palliation and late intracardiac repair.
Gutgesell HP, McNamara DG
Circulation 1975 Jan;51(1):32-8. doi: 10.1161/01.cir.51.1.32. PMID: 1109320

Recent clinical studies

Etiology

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
Technical Performance Score's Association With Arterial Switch Operation Outcomes.
Muter A, Evans HM, Gauvreau K, Colan S, Newburger J, Del Nido PJ, Nathan M
Ann Thorac Surg 2021 Apr;111(4):1367-1373. Epub 2020 Jun 27 doi: 10.1016/j.athoracsur.2020.05.057. PMID: 32603709
Management of 760-g Extremely Premature Infant With Dextro-Transposition of the Great Arteries.
Khazaal JR, Sowinski HA, Ratnasamy C, Vettukattil JJ, Haw MP
World J Pediatr Congenit Heart Surg 2020 Jul;11(4):518-519. doi: 10.1177/2150135120908777. PMID: 32645770
Transposition of the Great Arteries-Are We Doing Better? Correlating Outcome to Change in Renal Function Over 2 Decades of Arterial Switch Operation.
Shostak E, Dagan O, Hosh G, Amir G, Frenkel G, Schiller O
Pediatr Crit Care Med 2020 Sep;21(9):e782-e788. doi: 10.1097/PCC.0000000000002387. PMID: 32433443
Transposition of the great arteries. Results of treatment with early palliation and late intracardiac repair.
Gutgesell HP, McNamara DG
Circulation 1975 Jan;51(1):32-8. doi: 10.1161/01.cir.51.1.32. PMID: 1109320

Diagnosis

Double root translocation: Early experience on performance of translocated aortic and pulmonary root.
Agarwal V, Vaidyanathan S
Asian Cardiovasc Thorac Ann 2022 Oct;30(8):916-923. Epub 2022 Aug 15 doi: 10.1177/02184923221120751. PMID: 35971235
Technical Performance Score's Association With Arterial Switch Operation Outcomes.
Muter A, Evans HM, Gauvreau K, Colan S, Newburger J, Del Nido PJ, Nathan M
Ann Thorac Surg 2021 Apr;111(4):1367-1373. Epub 2020 Jun 27 doi: 10.1016/j.athoracsur.2020.05.057. PMID: 32603709
Population trends in prenatal detection of transposition of great arteries: impact of obstetric screening ultrasound guidelines.
Ravi P, Mills L, Fruitman D, Savard W, Colen T, Khoo N, Serrano-Lomelin J, Hornberger LK
Ultrasound Obstet Gynecol 2018 May;51(5):659-664. doi: 10.1002/uog.17496. PMID: 28436133
Dextro-transposition of the great arteries in a neonate with ventricular septal defect and pulmonary stenosis complicated by premature closure of the ductus arteriosus and possible pathophysiology of both defects.
Nakajima J, Kawakami T, Takeuchi K, Tsuchiya K
Pediatr Cardiol 2013;34(8):2009-12. Epub 2012 Nov 2 doi: 10.1007/s00246-012-0557-4. PMID: 23117331
Atriopulmonary anastomosis.
Kreutzer GO, Vargas FJ, Schlichter AJ, Laura JP, Suarez JC, Coronel AR, Kreutzer EA
J Thorac Cardiovasc Surg 1982 Mar;83(3):427-36. PMID: 7062754

Therapy

Being Overweight Is Related to Neoaortic Sinus Dilatation After Arterial Switch Operation.
Muneuchi J, Watanabe M, Sugitani Y, Doi H, Furuta T, Ezaki H, Kobayashi M, Ochiai Y
Tex Heart Inst J 2022 Sep 1;49(5) doi: 10.14503/THIJ-20-7508. PMID: 36260329Free PMC Article
Double root translocation: Early experience on performance of translocated aortic and pulmonary root.
Agarwal V, Vaidyanathan S
Asian Cardiovasc Thorac Ann 2022 Oct;30(8):916-923. Epub 2022 Aug 15 doi: 10.1177/02184923221120751. PMID: 35971235
Transposition of the Great Arteries-Are We Doing Better? Correlating Outcome to Change in Renal Function Over 2 Decades of Arterial Switch Operation.
Shostak E, Dagan O, Hosh G, Amir G, Frenkel G, Schiller O
Pediatr Crit Care Med 2020 Sep;21(9):e782-e788. doi: 10.1097/PCC.0000000000002387. PMID: 32433443
Strategic and operational aspects of a transfusion-free neonatal arterial switch operation.
Schweiger M, Dave H, Kelly J, Hübler M
Interact Cardiovasc Thorac Surg 2013 Jun;16(6):890-1. Epub 2013 Mar 4 doi: 10.1093/icvts/ivt010. PMID: 23460601Free PMC Article
Randomized trial of hematocrit 25% versus 35% during hypothermic cardiopulmonary bypass in infant heart surgery.
Newburger JW, Jonas RA, Soul J, Kussman BD, Bellinger DC, Laussen PC, Robertson R, Mayer JE Jr, del Nido PJ, Bacha EA, Forbess JM, Pigula F, Roth SJ, Visconti KJ, du Plessis AJ, Farrell DM, McGrath E, Rappaport LA, Wypij D
J Thorac Cardiovasc Surg 2008 Feb;135(2):347-54, 354.e1-4. doi: 10.1016/j.jtcvs.2007.01.051. PMID: 18242267

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
Technical Performance Score's Association With Arterial Switch Operation Outcomes.
Muter A, Evans HM, Gauvreau K, Colan S, Newburger J, Del Nido PJ, Nathan M
Ann Thorac Surg 2021 Apr;111(4):1367-1373. Epub 2020 Jun 27 doi: 10.1016/j.athoracsur.2020.05.057. PMID: 32603709
Transposition of the Great Arteries-Are We Doing Better? Correlating Outcome to Change in Renal Function Over 2 Decades of Arterial Switch Operation.
Shostak E, Dagan O, Hosh G, Amir G, Frenkel G, Schiller O
Pediatr Crit Care Med 2020 Sep;21(9):e782-e788. doi: 10.1097/PCC.0000000000002387. PMID: 32433443
Dextro-transposition of the great arteries in a neonate with ventricular septal defect and pulmonary stenosis complicated by premature closure of the ductus arteriosus and possible pathophysiology of both defects.
Nakajima J, Kawakami T, Takeuchi K, Tsuchiya K
Pediatr Cardiol 2013;34(8):2009-12. Epub 2012 Nov 2 doi: 10.1007/s00246-012-0557-4. PMID: 23117331
Atriopulmonary anastomosis.
Kreutzer GO, Vargas FJ, Schlichter AJ, Laura JP, Suarez JC, Coronel AR, Kreutzer EA
J Thorac Cardiovasc Surg 1982 Mar;83(3):427-36. PMID: 7062754

Clinical prediction guides

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
Technical Performance Score's Association With Arterial Switch Operation Outcomes.
Muter A, Evans HM, Gauvreau K, Colan S, Newburger J, Del Nido PJ, Nathan M
Ann Thorac Surg 2021 Apr;111(4):1367-1373. Epub 2020 Jun 27 doi: 10.1016/j.athoracsur.2020.05.057. PMID: 32603709
Population trends in prenatal detection of transposition of great arteries: impact of obstetric screening ultrasound guidelines.
Ravi P, Mills L, Fruitman D, Savard W, Colen T, Khoo N, Serrano-Lomelin J, Hornberger LK
Ultrasound Obstet Gynecol 2018 May;51(5):659-664. doi: 10.1002/uog.17496. PMID: 28436133
Randomized trial of hematocrit 25% versus 35% during hypothermic cardiopulmonary bypass in infant heart surgery.
Newburger JW, Jonas RA, Soul J, Kussman BD, Bellinger DC, Laussen PC, Robertson R, Mayer JE Jr, del Nido PJ, Bacha EA, Forbess JM, Pigula F, Roth SJ, Visconti KJ, du Plessis AJ, Farrell DM, McGrath E, Rappaport LA, Wypij D
J Thorac Cardiovasc Surg 2008 Feb;135(2):347-54, 354.e1-4. doi: 10.1016/j.jtcvs.2007.01.051. PMID: 18242267
Echocardiographic features of subpulmonic obstruction in dextro-transposition of the great vessels.
Nanda NC, Gramiak R, Manning JA, Lipchik EO
Circulation 1975 Mar;51(3):515-21. doi: 10.1161/01.cir.51.3.515. PMID: 1169132

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