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Double outlet right ventricle with subaortic or doubly committed ventricular septal defect with pulmonary stenosis

MedGen UID:
798712
Concept ID:
CN207265
Congenital Abnormality
Synonyms: DORV with subaortic or doubly committed VSD with pulmonary stenosis; DORV, Fallot type; double outlet right ventricle with subaortic or doubly committed ventricular septal defect with pulmonary stenosis; Double outlet right ventricle, Fallot type; double outlet right ventricle, Fallot type
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.
 
Monarch Initiative: MONDO:0020386
Orphanet: ORPHA99043

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDouble outlet right ventricle with subaortic or doubly committed ventricular septal defect with pulmonary stenosis

Professional guidelines

PubMed

Lagopoulos ME, Manlhiot C, McCrindle BW, Jaeggi ET, Friedberg MK, Nield LE
Am Heart J 2010 Oct;160(4):692-700. doi: 10.1016/j.ahj.2010.07.009. PMID: 20934564
Yeh HC, Wolf BS, Steinfield L, Baron MG
Radiology 1977 May;123(2):435-9. doi: 10.1148/123.2.435. PMID: 847217

Recent clinical studies

Etiology

Young AA, McBrien A, Caluseriu O, Haberer K, Wewala G, Eckersley L, Hornberger LK
Prenat Diagn 2021 Aug;41(9):1118-1126. Epub 2021 Jun 27 doi: 10.1002/pd.5979. PMID: 34118793
Bharucha T, Hlavacek AM, Spicer DE, Theocharis P, Anderson RH
Cardiol Young 2017 Jan;27(1):1-15. Epub 2016 Sep 19 doi: 10.1017/S1047951116001190. PMID: 27641710
Restivo A, Unolt M, Putotto C, Marino B
Anat Rec (Hoboken) 2013 Apr;296(4):559-63. Epub 2013 Feb 9 doi: 10.1002/ar.22657. PMID: 23401466
Lacour-Gayet F
Cardiol Young 2012 Dec;22(6):739-44. doi: 10.1017/S1047951112001564. PMID: 23331596
Lacour-Gayet F
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2002;5:163-72. doi: 10.1053/pcsu.2002.31491. PMID: 11994877

Diagnosis

Young AA, McBrien A, Caluseriu O, Haberer K, Wewala G, Eckersley L, Hornberger LK
Prenat Diagn 2021 Aug;41(9):1118-1126. Epub 2021 Jun 27 doi: 10.1002/pd.5979. PMID: 34118793
Ambra M, Nuri HA, Giuseppe P, Marasini M, Francesco S
World J Pediatr Congenit Heart Surg 2020 Jan;11(1):79-84. doi: 10.1177/2150135119885895. PMID: 31835981
Bharucha T, Hlavacek AM, Spicer DE, Theocharis P, Anderson RH
Cardiol Young 2017 Jan;27(1):1-15. Epub 2016 Sep 19 doi: 10.1017/S1047951116001190. PMID: 27641710
Restivo A, Unolt M, Putotto C, Marino B
Anat Rec (Hoboken) 2013 Apr;296(4):559-63. Epub 2013 Feb 9 doi: 10.1002/ar.22657. PMID: 23401466
Lacour-Gayet F
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2002;5:163-72. doi: 10.1053/pcsu.2002.31491. PMID: 11994877

Therapy

Yang XR, Wright JR Jr, Yu W, Langdon KD, Somerset D, Thomas MA
Birth Defects Res 2022 Jan 1;114(1):17-22. Epub 2021 Nov 13 doi: 10.1002/bdr2.1965. PMID: 34773723
Balaji S, Sreeram N
Indian Heart J 2017 Mar-Apr;69(2):240-243. Epub 2016 Dec 2 doi: 10.1016/j.ihj.2016.11.325. PMID: 28460773Free PMC Article
Villemain O, Bonnet D, Houyel L, Vergnat M, Ladouceur M, Lambert V, Jalal Z, Vouhé P, Belli E
Semin Thorac Cardiovasc Surg 2016 Spring;28(1):69-77. Epub 2016 May 11 doi: 10.1053/j.semtcvs.2016.01.007. PMID: 27568139
Li S, Ma K, Hu S, Hua Z, Yang K, Yan J, Chen Q
J Thorac Cardiovasc Surg 2014 Sep;148(3):817-24. Epub 2014 Jun 13 doi: 10.1016/j.jtcvs.2014.06.020. PMID: 24997522
Alsoufi B, Cai S, Williams WG, Coles JG, Caldarone CA, Redington AM, Van Arsdell GS
Eur J Cardiothorac Surg 2008 Feb;33(2):244-50. Epub 2007 Dec 21 doi: 10.1016/j.ejcts.2007.11.017. PMID: 18082414

Prognosis

Young AA, McBrien A, Caluseriu O, Haberer K, Wewala G, Eckersley L, Hornberger LK
Prenat Diagn 2021 Aug;41(9):1118-1126. Epub 2021 Jun 27 doi: 10.1002/pd.5979. PMID: 34118793
Ambra M, Nuri HA, Giuseppe P, Marasini M, Francesco S
World J Pediatr Congenit Heart Surg 2020 Jan;11(1):79-84. doi: 10.1177/2150135119885895. PMID: 31835981
Restivo A, Unolt M, Putotto C, Marino B
Anat Rec (Hoboken) 2013 Apr;296(4):559-63. Epub 2013 Feb 9 doi: 10.1002/ar.22657. PMID: 23401466
Lacour-Gayet F
Cardiol Young 2012 Dec;22(6):739-44. doi: 10.1017/S1047951112001564. PMID: 23331596
Gabriels C, Gewillig M, Meyns B, Troost E, Van De Bruaene A, Van Damme S, Budts W
Cardiology 2011;120(3):149-56. Epub 2011 Dec 23 doi: 10.1159/000334427. PMID: 22205053

Clinical prediction guides

Young AA, McBrien A, Caluseriu O, Haberer K, Wewala G, Eckersley L, Hornberger LK
Prenat Diagn 2021 Aug;41(9):1118-1126. Epub 2021 Jun 27 doi: 10.1002/pd.5979. PMID: 34118793
Bharucha T, Hlavacek AM, Spicer DE, Theocharis P, Anderson RH
Cardiol Young 2017 Jan;27(1):1-15. Epub 2016 Sep 19 doi: 10.1017/S1047951116001190. PMID: 27641710
Restivo A, Unolt M, Putotto C, Marino B
Anat Rec (Hoboken) 2013 Apr;296(4):559-63. Epub 2013 Feb 9 doi: 10.1002/ar.22657. PMID: 23401466
Lacour-Gayet F
Cardiol Young 2012 Dec;22(6):739-44. doi: 10.1017/S1047951112001564. PMID: 23331596
Sridaromont S, Ritter DG, Feldt RH, Davis GD, Edwards JE
Mayo Clin Proc 1978 Sep;53(9):555-77. PMID: 682686

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