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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.
Non-Mendelian inheritance (Orphanet)
 
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

Impact of prenatal diagnosis and anatomical subtype on outcome in double outlet right ventricle.
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
Echocardiography of double outlet right ventricle: new diagnostic criteria.
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

Fetal double outlet right ventricle without heterotaxy syndrome: Diagnostic spectrum, associated extracardiac pathology and clinical outcomes.
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
How should we diagnose and differentiate hearts with double-outlet right ventricle?
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
Double-Outlet Right Ventricle With Noncommitted Ventricular Septal Defect and 2 Adequate Ventricles: Is Anatomical Repair Advantageous?
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
Double outlet right ventricle versus aortic dextroposition: morphologically distinct defects.
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
Biventricular repair of double outlet right ventricle with noncommitted ventricular septal defect.
Lacour-Gayet F
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2002;5:163-72. doi: 10.1053/pcsu.2002.31491. PMID: 11994877

Diagnosis

Fetal double outlet right ventricle without heterotaxy syndrome: Diagnostic spectrum, associated extracardiac pathology and clinical outcomes.
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
Double-Outlet Right Atrium: Review of a Rare Anomaly With an Exemplary Case.
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
How should we diagnose and differentiate hearts with double-outlet right ventricle?
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
Double outlet right ventricle versus aortic dextroposition: morphologically distinct defects.
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
Biventricular repair of double outlet right ventricle with noncommitted ventricular septal defect.
Lacour-Gayet F
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2002;5:163-72. doi: 10.1053/pcsu.2002.31491. PMID: 11994877

Therapy

Parietal bone agenesis and athelia in retinoic acid embryopathy: An expansion of the phenotype.
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
The development of pacing induced ventricular dysfunction is influenced by the underlying structural heart defect in children with congenital heart disease.
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
Double-Outlet Right Ventricle With Noncommitted Ventricular Septal Defect and 2 Adequate Ventricles: Is Anatomical Repair Advantageous?
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
Surgical outcomes of 380 patients with double outlet right ventricle who underwent biventricular repair.
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
Improved results with single-stage total correction of Taussig-Bing anomaly.
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

Fetal double outlet right ventricle without heterotaxy syndrome: Diagnostic spectrum, associated extracardiac pathology and clinical outcomes.
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
Double-Outlet Right Atrium: Review of a Rare Anomaly With an Exemplary Case.
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
Double outlet right ventricle versus aortic dextroposition: morphologically distinct defects.
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
The pathology of subaortic obstruction.
Anderson RH, Ho SY
Ann Thorac Surg 1998 Aug;66(2):644-8. doi: 10.1016/s0003-4975(98)00576-1. PMID: 9725445
Double-outlet right ventricle. Anatomic and angiocardiographic correlations.
Sridaromont S, Ritter DG, Feldt RH, Davis GD, Edwards JE
Mayo Clin Proc 1978 Sep;53(9):555-77. PMID: 682686

Clinical prediction guides

Fetal double outlet right ventricle without heterotaxy syndrome: Diagnostic spectrum, associated extracardiac pathology and clinical outcomes.
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
How should we diagnose and differentiate hearts with double-outlet right ventricle?
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
Double outlet right ventricle versus aortic dextroposition: morphologically distinct defects.
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
Complexity stratification of the arterial switch operation: a second learning curve.
Lacour-Gayet F
Cardiol Young 2012 Dec;22(6):739-44. doi: 10.1017/S1047951112001564. PMID: 23331596
Double-outlet right ventricle. Anatomic and angiocardiographic correlations.
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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