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Double outlet right ventricle with doubly committed ventricular septal defect without pulmonary stenosis

MedGen UID:
868837
•Concept ID:
C4023247
•
Congenital Abnormality
Synonyms: DORV with doubly committed VSD; Double outlet right ventricle, doubly committed ventricular septal defect
 
HPO: HP:0011652

Definition

A double outlet right ventricle with a subaortic ventritricular 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 not 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 without pulmonary stenosis

Recent clinical studies

Etiology

Determinants of repair type, reintervention, and mortality in 393 children with double-outlet right ventricle.
Bradley TJ, Karamlou T, Kulik A, Mitrovic B, Vigneswaran T, Jaffer S, Glasgow PD, Williams WG, Van Arsdell GS, McCrindle BW
J Thorac Cardiovasc Surg 2007 Oct;134(4):967-973.e6. doi: 10.1016/j.jtcvs.2007.05.061. PMID: 17903515
Repair of double outlet right ventricle with doubly-committed ventricular septal defect.
Uemura H, Yagihara T, Kadohama T, Kawahira Y, Yoshikawa Y
Cardiol Young 2001 Jul;11(4):415-9. doi: 10.1017/s1047951101000531. PMID: 11558951
Biventricular repair for double-outlet right ventricle. Results and long-term follow-up.
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
Surgical management of double-outlet right ventricle.
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
Repair of double-outlet right ventricle. An analysis of 62 cases.
Stewart RW, Kirklin JW, Pacifico AD, Blackstone EH, Bargeron LM Jr
J Thorac Cardiovasc Surg 1979 Oct;78(4):502-14. PMID: 480960

Prognosis

Repair of double outlet right ventricle with doubly-committed ventricular septal defect.
Uemura H, Yagihara T, Kadohama T, Kawahira Y, Yoshikawa Y
Cardiol Young 2001 Jul;11(4):415-9. doi: 10.1017/s1047951101000531. PMID: 11558951
Biventricular repair for double-outlet right ventricle. Results and long-term follow-up.
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
Subaortic obstruction in double outlet right ventricles. Surgical considerations for anatomic repair.
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
Current risks and protocols for operations for double-outlet right ventricle. Derivation from an 18 year experience.
Kirklin JW, Pacifico AD, Blackstone EH, Kirklin JK, Bargeron LM Jr
J Thorac Cardiovasc Surg 1986 Nov;92(5):913-30. PMID: 2430144
Surgical management of double-outlet right ventricle.
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

Compression of an anomalous single coronary artery from pulmonary artery by banding.
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
Surgical management of double-outlet right ventricle.
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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