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Double outlet right ventricle(DORV)

MedGen UID:
41649
Concept ID:
C0013069
Congenital Abnormality
Synonyms: DORV
SNOMED CT: Double outlet right ventricle (7484005); Origin of both great vessels from right ventricle (7484005); Dextratransposition of aorta (7484005); Transposition of great vessels, interventricular septal defect AND overriding aorta (7484005); DORV - Double outlet right ventricle (7484005)
 
OMIM®: 217095
Orphanet: ORPHA3426

Definition

A rare congenital cardiovascular abnormality in which both the aorta and the pulmonary artery arise from the right ventricle. [from NCI]

Recent clinical studies

Etiology

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. [Epub ahead of print] PMID: 24997522
Sabati AA, Wong PC, Randolph L, Pruetz JD
Congenit Heart Dis 2014 May-Jun;9(3):E98-E104. Epub 2013 May 22 doi: 10.1111/chd.12089. [Epub ahead of print] PMID: 23701730
Zidere V, Pushparajah K, Allan LD, Simpson JM
Ultrasound Obstet Gynecol 2013 Oct;42(4):421-5. Epub 2013 Sep 2 doi: 10.1002/uog.12512. [Epub ahead of print] PMID: 23681839
Raju V, Burkhart HM, Rigelman Hedberg N, Eidem BW, Li Z, Connolly H, Schaff HV, Dearani JA
Ann Thorac Surg 2013 Jun;95(6):2079-84; discussion 2084-5. Epub 2013 Apr 18 doi: 10.1016/j.athoracsur.2013.02.016. [Epub ahead of print] PMID: 23602064
Lin CH, Huddleston C, Balzer DT
Pediatr Cardiol 2013 Mar;34(3):743-7. Epub 2012 May 12 doi: 10.1007/s00246-012-0337-1. [Epub ahead of print] PMID: 22580772Free PMC Article

Diagnosis

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. [Epub ahead of print] PMID: 24997522
Özgür S, Ceylan Ö, Doğan V, Örün UA
Turk Kardiyol Dern Ars 2014 Mar;42(2):190-3. PMID: 24643154
Keswani A, Verma A, Dann K, Ventura L, Lucas V, Shah S, Ramee S
Congenit Heart Dis 2014 Sep-Oct;9(5):E153-7. Epub 2013 Jul 19 doi: 10.1111/chd.12114. [Epub ahead of print] PMID: 23865720
Sabati AA, Wong PC, Randolph L, Pruetz JD
Congenit Heart Dis 2014 May-Jun;9(3):E98-E104. Epub 2013 May 22 doi: 10.1111/chd.12089. [Epub ahead of print] PMID: 23701730
McMahon CJ, Walsh KP
Catheter Cardiovasc Interv 2013 Dec 1;82(7):1164-7. Epub 2013 Mar 14 doi: 10.1002/ccd.24852. [Epub ahead of print] PMID: 23404713

Therapy

Beegle RD, Mastin ST, Chandran A
J Cardiovasc Comput Tomogr 2014 Sep-Oct;8(5):401-3. Epub 2014 May 29 doi: 10.1016/j.jcct.2014.05.003. [Epub ahead of print] PMID: 24998091
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. [Epub ahead of print] PMID: 24997522
Zidere V, Pushparajah K, Allan LD, Simpson JM
Ultrasound Obstet Gynecol 2013 Oct;42(4):421-5. Epub 2013 Sep 2 doi: 10.1002/uog.12512. [Epub ahead of print] PMID: 23681839
Raju V, Burkhart HM, Rigelman Hedberg N, Eidem BW, Li Z, Connolly H, Schaff HV, Dearani JA
Ann Thorac Surg 2013 Jun;95(6):2079-84; discussion 2084-5. Epub 2013 Apr 18 doi: 10.1016/j.athoracsur.2013.02.016. [Epub ahead of print] PMID: 23602064
Ortega M, Merino JL, Blanco FV, Gutiérrez-Larraya F
J Cardiovasc Electrophysiol 2012 Jul;23(7):781-3. Epub 2012 Feb 9 doi: 10.1111/j.1540-8167.2011.02282.x. [Epub ahead of print] PMID: 22320348

Prognosis

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. [Epub ahead of print] PMID: 24997522
Sabati AA, Wong PC, Randolph L, Pruetz JD
Congenit Heart Dis 2014 May-Jun;9(3):E98-E104. Epub 2013 May 22 doi: 10.1111/chd.12089. [Epub ahead of print] PMID: 23701730
Zidere V, Pushparajah K, Allan LD, Simpson JM
Ultrasound Obstet Gynecol 2013 Oct;42(4):421-5. Epub 2013 Sep 2 doi: 10.1002/uog.12512. [Epub ahead of print] PMID: 23681839
Raju V, Burkhart HM, Rigelman Hedberg N, Eidem BW, Li Z, Connolly H, Schaff HV, Dearani JA
Ann Thorac Surg 2013 Jun;95(6):2079-84; discussion 2084-5. Epub 2013 Apr 18 doi: 10.1016/j.athoracsur.2013.02.016. [Epub ahead of print] PMID: 23602064
Lin CH, Huddleston C, Balzer DT
Pediatr Cardiol 2013 Mar;34(3):743-7. Epub 2012 May 12 doi: 10.1007/s00246-012-0337-1. [Epub ahead of print] PMID: 22580772Free PMC Article

Clinical prediction guides

Capuani A
J Cardiothorac Surg 2014 Apr 21;9:71. doi: 10.1186/1749-8090-9-71. [Epub ahead of print] PMID: 24750982Free PMC Article
Özgür S, Ceylan Ö, Doğan V, Örün UA
Turk Kardiyol Dern Ars 2014 Mar;42(2):190-3. PMID: 24643154
Keswani A, Verma A, Dann K, Ventura L, Lucas V, Shah S, Ramee S
Congenit Heart Dis 2014 Sep-Oct;9(5):E153-7. Epub 2013 Jul 19 doi: 10.1111/chd.12114. [Epub ahead of print] PMID: 23865720
Zidere V, Pushparajah K, Allan LD, Simpson JM
Ultrasound Obstet Gynecol 2013 Oct;42(4):421-5. Epub 2013 Sep 2 doi: 10.1002/uog.12512. [Epub ahead of print] PMID: 23681839
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. [Epub ahead of print] PMID: 23401466

Recent systematic reviews

Brandler WM, Morris AP, Evans DM, Scerri TS, Kemp JP, Timpson NJ, St Pourcain B, Smith GD, Ring SM, Stein J, Monaco AP, Talcott JB, Fisher SE, Webber C, Paracchini S
PLoS Genet 2013;9(9):e1003751. Epub 2013 Sep 12 doi: 10.1371/journal.pgen.1003751. PMID: 24068947Free PMC Article
Siddiqui MT, Hasan A, Mohsin S, Hamid M, Amanullah MM
J Pak Med Assoc 2012 Oct;62(10):1113-7. PMID: 23866464
Hubail ZJ, Ramaciotti C
Congenit Heart Dis 2007 Nov-Dec;2(6):421-3. doi: 10.1111/j.1747-0803.2007.00135.x. PMID: 18377435

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