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Double outlet right ventricle with subpulmonary ventricular septal defect

MedGen UID:
365502
•Concept ID:
C1956412
•
Congenital Abnormality
Synonym: Double Outlet Right Ventricle, Subpulmonary VSD
SNOMED CT: Double outlet right ventricle with subpulmonary ventricular septal defect (448794008)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
•Concept ID:
C0600599
•
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Non-Mendelian inheritance (Orphanet)
 
Monarch Initiative: MONDO:0020387
Orphanet: ORPHA99045

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDouble outlet right ventricle with subpulmonary ventricular septal defect

Professional guidelines

PubMed

Prenatal Diagnosis and Postnatal Outcome of Eight Cases with Criss-Cross Heart - A Multicenter Case Series.
Vorisek CN, Kurkevych A, Kuhn V, Stessig R, Ritgen J, Degenhardt J, Enzensberger C, Wolter A, Götte M, Khalil M, Akintürk H, Axt-Fliedner R
Ultraschall Med 2022 Dec;43(6):e90-e97. Epub 2020 Jul 16 doi: 10.1055/a-1205-0289. PMID: 32674186
Challenges of classifying double outlet right ventricle: importance for genotype-phenotype analyses.
Silvestri LM, Scarabotti A, Marino B
Am J Med Genet A 2014 May;164A(5):1340. Epub 2014 Mar 24 doi: 10.1002/ajmg.a.36448. PMID: 24664979
Prenatal diagnosis of a fetal double-outlet right ventricle with tricuspid valve atresia, a straddling mitral valve, and a hypoplastic left ventricle.
Wu H, Cai A, Zhang Y, Sun W, Wang Y
J Ultrasound Med 2012 Apr;31(4):654-6. doi: 10.7863/jum.2012.31.4.654. PMID: 22441924

Recent clinical studies

Etiology

Anatomical Classifications of the Coronary Arteries in Complete Transposition of the Great Arteries and Double Outlet Right Ventricle with Subpulmonary Ventricular Septal Defect.
Wang C, Chen S, Zhang H, Liu J, Xu Z, Zheng J, Yan Q, Huang H, Huang M
Thorac Cardiovasc Surg 2017 Jan;65(1):26-30. Epub 2016 Mar 2 doi: 10.1055/s-0036-1571828. PMID: 26935177
Double-root translocation for double-outlet right ventricle with noncommitted ventricular septal defect or double-outlet right ventricle with subpulmonary ventricular septal defect associated with pulmonary stenosis: an optimized solution.
Hu S, Xie Y, Li S, Wang X, Yan F, Li Y, Hua Z, Li Y
Ann Thorac Surg 2010 May;89(5):1360-5. doi: 10.1016/j.athoracsur.2010.02.007. PMID: 20417745
Ventricular outflow tracts after Kawashima intraventricular rerouting for double outlet right ventricle with subpulmonary ventricular septal defect.
Kawahira Y, Yagihara T, Uemura H, Ishizaka T, Yoshikawa Y, Yoshizumi K, Kitamura S
Eur J Cardiothorac Surg 1999 Jul;16(1):26-31. doi: 10.1016/s1010-7940(99)00135-9. PMID: 10456398
Early results for anatomic correction of transposition of the great arteries and for double-outlet right ventricle with subpulmonary ventricular septal defect.
Brawn WJ, Mee RB
J Thorac Cardiovasc Surg 1988 Feb;95(2):230-8. PMID: 3339890
Results of the Damus-Stansel-Kaye procedure for transposition of the great arteries and for double-outlet right ventricle with subpulmonary ventricular septal defect.
Ceithaml EL, Puga FJ, Danielson GK, McGoon DC, Ritter DG
Ann Thorac Surg 1984 Nov;38(5):433-7. doi: 10.1016/s0003-4975(10)64180-x. PMID: 6497473

Diagnosis

Anatomical Classifications of the Coronary Arteries in Complete Transposition of the Great Arteries and Double Outlet Right Ventricle with Subpulmonary Ventricular Septal Defect.
Wang C, Chen S, Zhang H, Liu J, Xu Z, Zheng J, Yan Q, Huang H, Huang M
Thorac Cardiovasc Surg 2017 Jan;65(1):26-30. Epub 2016 Mar 2 doi: 10.1055/s-0036-1571828. PMID: 26935177
Current expectations of the arterial switch operation in a small volume center: a 20-year, single-center experience.
Shim MS, Jun TG, Yang JH, Park PW, Kang IS, Huh J, Song JY
J Cardiothorac Surg 2016 Feb 24;11:34. doi: 10.1186/s13019-016-0428-9. PMID: 26911711Free PMC Article
Early prenatal detection of double outlet right ventricle by echocardiography.
Stewart PA, Wladimiroff JW, Becker AE
Br Heart J 1985 Sep;54(3):340-2. doi: 10.1136/hrt.54.3.340. PMID: 4041305Free PMC Article

Therapy

Anatomical Classifications of the Coronary Arteries in Complete Transposition of the Great Arteries and Double Outlet Right Ventricle with Subpulmonary Ventricular Septal Defect.
Wang C, Chen S, Zhang H, Liu J, Xu Z, Zheng J, Yan Q, Huang H, Huang M
Thorac Cardiovasc Surg 2017 Jan;65(1):26-30. Epub 2016 Mar 2 doi: 10.1055/s-0036-1571828. PMID: 26935177
Current expectations of the arterial switch operation in a small volume center: a 20-year, single-center experience.
Shim MS, Jun TG, Yang JH, Park PW, Kang IS, Huh J, Song JY
J Cardiothorac Surg 2016 Feb 24;11:34. doi: 10.1186/s13019-016-0428-9. PMID: 26911711Free PMC Article
Risk factors for neo-aortic root enlargement and aortic regurgitation following arterial switch operation.
McMahon CJ, Ravekes WJ, Smith EO, Denfield SW, Pignatelli RH, Altman CA, Ayres NA
Pediatr Cardiol 2004 Jul-Aug;25(4):329-35. doi: 10.1007/s00246-003-0483-6. PMID: 14727099

Prognosis

Anatomical Classifications of the Coronary Arteries in Complete Transposition of the Great Arteries and Double Outlet Right Ventricle with Subpulmonary Ventricular Septal Defect.
Wang C, Chen S, Zhang H, Liu J, Xu Z, Zheng J, Yan Q, Huang H, Huang M
Thorac Cardiovasc Surg 2017 Jan;65(1):26-30. Epub 2016 Mar 2 doi: 10.1055/s-0036-1571828. PMID: 26935177
Current expectations of the arterial switch operation in a small volume center: a 20-year, single-center experience.
Shim MS, Jun TG, Yang JH, Park PW, Kang IS, Huh J, Song JY
J Cardiothorac Surg 2016 Feb 24;11:34. doi: 10.1186/s13019-016-0428-9. PMID: 26911711Free PMC Article
Double-root translocation for double-outlet right ventricle with noncommitted ventricular septal defect or double-outlet right ventricle with subpulmonary ventricular septal defect associated with pulmonary stenosis: an optimized solution.
Hu S, Xie Y, Li S, Wang X, Yan F, Li Y, Hua Z, Li Y
Ann Thorac Surg 2010 May;89(5):1360-5. doi: 10.1016/j.athoracsur.2010.02.007. PMID: 20417745
Early results for anatomic correction of transposition of the great arteries and for double-outlet right ventricle with subpulmonary ventricular septal defect.
Brawn WJ, Mee RB
J Thorac Cardiovasc Surg 1988 Feb;95(2):230-8. PMID: 3339890
Results of the Damus-Stansel-Kaye procedure for transposition of the great arteries and for double-outlet right ventricle with subpulmonary ventricular septal defect.
Ceithaml EL, Puga FJ, Danielson GK, McGoon DC, Ritter DG
Ann Thorac Surg 1984 Nov;38(5):433-7. doi: 10.1016/s0003-4975(10)64180-x. PMID: 6497473

Clinical prediction guides

Anatomical Classifications of the Coronary Arteries in Complete Transposition of the Great Arteries and Double Outlet Right Ventricle with Subpulmonary Ventricular Septal Defect.
Wang C, Chen S, Zhang H, Liu J, Xu Z, Zheng J, Yan Q, Huang H, Huang M
Thorac Cardiovasc Surg 2017 Jan;65(1):26-30. Epub 2016 Mar 2 doi: 10.1055/s-0036-1571828. PMID: 26935177
Risk factors for neo-aortic root enlargement and aortic regurgitation following arterial switch operation.
McMahon CJ, Ravekes WJ, Smith EO, Denfield SW, Pignatelli RH, Altman CA, Ayres NA
Pediatr Cardiol 2004 Jul-Aug;25(4):329-35. doi: 10.1007/s00246-003-0483-6. PMID: 14727099
The arterial switch operation. An eight-year experience.
Quaegebeur JM, Rohmer J, Ottenkamp J, Buis T, Kirklin JW, Blackstone EH, Brom AG
J Thorac Cardiovasc Surg 1986 Sep;92(3 Pt 1):361-84. PMID: 3747568

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