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Double outlet right ventricle with non-committed subpulmonary ventricular septal defect

MedGen UID:
365501
•Concept ID:
C1956410
•
Congenital Abnormality
Synonym: Double Outlet Right Ventricle, Noncommitted VSD
SNOMED CT: Double outlet right ventricle with noncommitted ventricular septal defect (253299006)
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:0020388
Orphanet: ORPHA99046

Definition

A subtype with ventricular septal defect clearly away from the semilunar valves of the AORTA and the PULMONARY ARTERY. [from MeSH]

Term Hierarchy

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

Recent clinical studies

Etiology

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
The surgical anatomy of ventricular septal defect part IV: double outlet ventricle.
Anderson RH, Ho SY, Wilcox BR
J Card Surg 1996 Jan-Feb;11(1):2-11. doi: 10.1111/j.1540-8191.1996.tb00002.x. PMID: 8775329

Diagnosis

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
Usefulness of 3D reconstructed computed tomography imaging for double outlet right ventricle.
Chen SJ, Lin MT, Liu KL, Chang CI, Chen HY, Wang JK, Lee WJ, Tsang YM, Li YW
J Formos Med Assoc 2008 May;107(5):371-80. doi: 10.1016/S0929-6646(08)60102-3. PMID: 18492621
Double outlet right ventricle with discordant atrioventricular connexion: an angiographic analysis of 19 cases.
Battistessa S, Soto B
Int J Cardiol 1990 May;27(2):253-63; discussion 265-7. doi: 10.1016/0167-5273(90)90167-4. PMID: 2365513

Clinical prediction guides

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
Usefulness of 3D reconstructed computed tomography imaging for double outlet right ventricle.
Chen SJ, Lin MT, Liu KL, Chang CI, Chen HY, Wang JK, Lee WJ, Tsang YM, Li YW
J Formos Med Assoc 2008 May;107(5):371-80. doi: 10.1016/S0929-6646(08)60102-3. PMID: 18492621

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