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

MedGen UID:
489000
Concept ID:
C3165091
Congenital Abnormality
Synonym: DORV with doubly committed VSD and pulmonary stenosis
SNOMED CT: Double outlet right ventricle with doubly committed ventricular septal defect and pulmonary stenosis (448516008)
 
HPO: HP:0011651

Definition

A double outlet right ventricle with a subaortic ventricular septal defect (a hole between the two bottom chambers (ventricles) of the heart), that extends anterosuperiorly and are closely related to the pulmonary artery as well, are considered to be doubly committed. There is associated pulmonary stenosis, the abnormal narrowing or constriction of the pulmonary artery, in the main pulmonary artery and/or in the left or right pulmonary artery branches. [from HPO]

Term Hierarchy

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

Recent clinical studies

Etiology

Uemura H, Yagihara T, Kadohama T, Kawahira Y, Yoshikawa Y
Cardiol Young 2001 Jul;11(4):415-9. doi: 10.1017/s1047951101000531. PMID: 11558951
Belli E, Serraf A, Lacour-Gayet F, Prodan S, Piot D, Losay J, Petit J, Bruniaux J, Planché C
Circulation 1998 Nov 10;98(19 Suppl):II360-5; discussion II365-7. PMID: 9852927
Tchervenkov CI, Marelli D, Béland MJ, Gibbons JE, Paquet M, Dobell AR
Ann Thorac Surg 1995 Dec;60(6 Suppl):S610-3. doi: 10.1016/0003-4975(95)00855-1. PMID: 8604947
Serraf A, Lacour-Gayet F, Houyel L, Bruniaux J, Uva MS, Roux D, Piot D, Planché C
Circulation 1993 Nov;88(5 Pt 2):II177-82. PMID: 8222151
Stewart RW, Kirklin JW, Pacifico AD, Blackstone EH, Bargeron LM Jr
J Thorac Cardiovasc Surg 1979 Oct;78(4):502-14. PMID: 480960

Prognosis

Uemura H, Yagihara T, Kadohama T, Kawahira Y, Yoshikawa Y
Cardiol Young 2001 Jul;11(4):415-9. doi: 10.1017/s1047951101000531. PMID: 11558951
Belli E, Serraf A, Lacour-Gayet F, Prodan S, Piot D, Losay J, Petit J, Bruniaux J, Planché C
Circulation 1998 Nov 10;98(19 Suppl):II360-5; discussion II365-7. PMID: 9852927
Serraf A, Lacour-Gayet F, Houyel L, Bruniaux J, Uva MS, Roux D, Piot D, Planché C
Circulation 1993 Nov;88(5 Pt 2):II177-82. PMID: 8222151
Kirklin JW, Pacifico AD, Blackstone EH, Kirklin JK, Bargeron LM Jr
J Thorac Cardiovasc Surg 1986 Nov;92(5):913-30. PMID: 2430144
Mazzucco A, Faggian G, Stellin G, Bortolotti U, Livi U, Rizzoli G, Gallucci V
J Thorac Cardiovasc Surg 1985 Jul;90(1):29-34. PMID: 2409406

Clinical prediction guides

Kawamura A, Oshima Y, Maruo A, Matsuhisa H
Eur J Cardiothorac Surg 2012 Apr;41(4):e59-61. Epub 2012 Jan 13 doi: 10.1093/ejcts/ezr269. PMID: 22246964
Mazzucco A, Faggian G, Stellin G, Bortolotti U, Livi U, Rizzoli G, Gallucci V
J Thorac Cardiovasc Surg 1985 Jul;90(1):29-34. PMID: 2409406

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