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

MedGen UID:
1843284
•Concept ID:
C5680070
•
Congenital Abnormality
Synonyms: DORV with subaortic or doubly committed VSD; double outlet right ventricle with subaortic or doubly committed ventricular septal defect
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:0018498
Orphanet: ORPHA423693

Term Hierarchy

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

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
Surgical management of ventricular septal defect with aortic valve prolapse: clinical considerations and results.
Brauner R, Birk E, Sahar G, Blieden L, Vidne BA
Eur J Cardiothorac Surg 1995;9(6):315-9. doi: 10.1016/s1010-7940(05)80189-7. PMID: 7546804
Doubly committed subarterial ventricular septal defect: new morphological criteria with echocardiographic and angiocardiographic correlation.
Griffin ML, Sullivan ID, Anderson RH, Macartney FJ
Br Heart J 1988 Apr;59(4):474-9. doi: 10.1136/hrt.59.4.474. PMID: 3370182Free PMC Article

Recent clinical studies

Etiology

Neonatal repair of atypical double outlet right ventricle.
Yokoyama S, Fukuba R, Tonomura R, Mitani K, Uemura H
J Card Surg 2022 Jul;37(7):2124-2126. Epub 2022 Apr 5 doi: 10.1111/jocs.16488. PMID: 35384047
Doubly committed subarterial ventricular septal defect closure through tricuspid approach: a clinical analysis.
Wang G, Liu R, Ma K, Zhang B, Pang K, Qi L, Hua Z, Li S
Cardiol Young 2021 May;31(5):799-803. Epub 2021 Jan 28 doi: 10.1017/S1047951120004643. PMID: 33504385
Percutaneous-perventricular device closure of ventricular septal defect: mid-term follow-up.
Wang L, Xie L, Ruan W, Li T, Gan C, Lin K
BMC Surg 2020 Sep 18;20(1):208. doi: 10.1186/s12893-020-00854-0. PMID: 32948172Free PMC Article
Doubly Committed Subarterial Ventricular Septal Defects Closure Using Minimal Mid-Partial Sternotomy.
Huang C, Yu T, Zhang X
Ann Thorac Cardiovasc Surg 2019 Jun 20;25(3):172-175. Epub 2018 Dec 11 doi: 10.5761/atcs.oa.18-00184. PMID: 30541999Free PMC Article
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

Diagnosis

Outcomes of closure of doubly committed subarterial ventricular septal defects in adults.
Chen Z, Chen W, Chen H, Liao Z, Chen Q, Chen L
Cardiol Young 2020 May;30(5):599-606. Epub 2020 Apr 20 doi: 10.1017/S1047951120000530. PMID: 32308178
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
Doubly Committed Subarterial Ventricular Septal Defects Closure Using Minimal Mid-Partial Sternotomy.
Huang C, Yu T, Zhang X
Ann Thorac Cardiovasc Surg 2019 Jun 20;25(3):172-175. Epub 2018 Dec 11 doi: 10.5761/atcs.oa.18-00184. PMID: 30541999Free PMC Article
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
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

Outcomes of closure of doubly committed subarterial ventricular septal defects in adults.
Chen Z, Chen W, Chen H, Liao Z, Chen Q, Chen L
Cardiol Young 2020 May;30(5):599-606. Epub 2020 Apr 20 doi: 10.1017/S1047951120000530. PMID: 32308178
A meta-analysis of perventricular device closure of doubly committed subarterial ventricular septal defects.
Huang JS, Sun KP, Huang ST, Chen Q, Chen LW, Kuo YR
J Cardiothorac Surg 2020 Jan 28;15(1):28. doi: 10.1186/s13019-020-1062-0. PMID: 31992348Free PMC Article
Transcatheter closure of doubly committed subarterial ventricular septal defects with the Amplatzer ductal occluder: initial experience.
Huang Y, Yan X, Lu L, Fang J, Sun X, Zhong L, Li J, Chen L
Cardiol Young 2019 May;29(5):570-575. Epub 2019 Apr 10 doi: 10.1017/S104795111900009X. PMID: 30968785
Doubly Committed Subarterial Ventricular Septal Defects Closure Using Minimal Mid-Partial Sternotomy.
Huang C, Yu T, Zhang X
Ann Thorac Cardiovasc Surg 2019 Jun 20;25(3):172-175. Epub 2018 Dec 11 doi: 10.5761/atcs.oa.18-00184. PMID: 30541999Free 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

Prognosis

Doubly committed subarterial ventricular septal defect closure through tricuspid approach: a clinical analysis.
Wang G, Liu R, Ma K, Zhang B, Pang K, Qi L, Hua Z, Li S
Cardiol Young 2021 May;31(5):799-803. Epub 2021 Jan 28 doi: 10.1017/S1047951120004643. PMID: 33504385
Percutaneous-perventricular device closure of ventricular septal defect: mid-term follow-up.
Wang L, Xie L, Ruan W, Li T, Gan C, Lin K
BMC Surg 2020 Sep 18;20(1):208. doi: 10.1186/s12893-020-00854-0. PMID: 32948172Free PMC Article
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
Doubly Committed Subarterial Ventricular Septal Defects Closure Using Minimal Mid-Partial Sternotomy.
Huang C, Yu T, Zhang X
Ann Thorac Cardiovasc Surg 2019 Jun 20;25(3):172-175. Epub 2018 Dec 11 doi: 10.5761/atcs.oa.18-00184. PMID: 30541999Free PMC Article
Doubly committed ventricular septal defect closure using eccentric occluder via ultraminimal incision.
Yu J, Ma L, Ye J, Zhang Z, Li J, Yu J, Jiang G
Eur J Cardiothorac Surg 2017 Oct 1;52(4):805-809. doi: 10.1093/ejcts/ezx269. PMID: 29156020

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

Recent systematic reviews

A systematic review on the efficacy and safety of transcatheter device closure of ventricular septal defects (VSD).
Yang L, Tai BC, Khin LW, Quek SC
J Interv Cardiol 2014 Jun;27(3):260-72. Epub 2014 Apr 29 doi: 10.1111/joic.12121. PMID: 24773223

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