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Double outlet left ventricle(DOLV)

MedGen UID:
120558
Concept ID:
C0265809
Congenital Abnormality
Synonym: DOLV
SNOMED CT: DOLV - Double outlet left ventricle (7368005); Double outlet left ventricle (7368005); Origin of both great vessels from left ventricle (7368005)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
HPO: HP:0011581
Monarch Initiative: MONDO:0018090
Orphanet: ORPHA3427

Definition

A congenital defect of heart development characterized by origin of both pulmonary artery and aorta from the morphological left ventricle. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDouble outlet left ventricle
Follow this link to review classifications for Double outlet left ventricle in Orphanet.

Conditions with this feature

Pancreatic hypoplasia-diabetes-congenital heart disease syndrome
MedGen UID:
860891
Concept ID:
C4012454
Congenital Abnormality
A rare, syndromic diabetes mellitus characterized by partial pancreatic agenesis, diabetes mellitus, and heart anomalies (including transposition of the great vessels, ventricular or atrial septal defects, pulmonary stenosis, or patent ductus arteriosis).

Professional guidelines

PubMed

Brandt PW, Calder AL, Barratt-Boyes BG, Neutze JM
Am J Cardiol 1976 Dec;38(7):897-909. doi: 10.1016/0002-9149(76)90803-1. PMID: 998525

Recent clinical studies

Etiology

Xu Z, Semple T, Gu H, McCarthy KP, Yen Ho S, Li W
Heart 2023 May 26;109(12):905-912. doi: 10.1136/heartjnl-2022-321955. PMID: 36539269
Raja J, Menon S, Ramanan S, Dutta Baruah S, Devarakonda BV, Gopalakrishnan A, Dharan BS
J Card Surg 2020 Aug;35(8):1865-1870. Epub 2020 Jul 11 doi: 10.1111/jocs.14768. PMID: 32652736
Luciani GB, De Rita F, Lucchese G, Barozzi L, Rossetti L, Faggian G, Mazzucco A
J Cardiovasc Med (Hagerstown) 2017 May;18(5):311-317. doi: 10.2459/JCM.0000000000000101. PMID: 25050529
Imai-Compton C, Elmi M, Manlhiot C, Floh AA, Golding F, Williams WG, McCrindle BW
Congenit Heart Dis 2010 Nov-Dec;5(6):532-6. doi: 10.1111/j.1747-0803.2010.00456.x. PMID: 21106011
Lopes LM, Rangel PI, Soraggi AM, Furlanetto BH, Furlanetto G
Arq Bras Cardiol 2001 Jun;76(6):511-6. doi: 10.1590/s0066-782x2001000600008. PMID: 11449296

Diagnosis

Xu Z, Semple T, Gu H, McCarthy KP, Yen Ho S, Li W
Heart 2023 May 26;109(12):905-912. doi: 10.1136/heartjnl-2022-321955. PMID: 36539269
Alifu A, Wang H, Su Y, Chen R
Medicine (Baltimore) 2022 Nov 25;101(47):e32070. doi: 10.1097/MD.0000000000032070. PMID: 36451474Free PMC Article
Sakai W, Asai H, Ebuoka N
Cardiol Young 2022 Nov;32(11):1864-1865. Epub 2022 Feb 28 doi: 10.1017/S1047951122000786. PMID: 35225204
Sun F, Fan M, Huang L, Ren W, Li D
J Clin Ultrasound 2018 May;46(4):265-268. Epub 2017 Sep 7 doi: 10.1002/jcu.22520. PMID: 28881023
Tchervenkov CI, Walters HL 3rd, Chu VF
Ann Thorac Surg 2000 Apr;69(4 Suppl):S264-9. doi: 10.1016/s0003-4975(99)01281-3. PMID: 10798434

Therapy

Postalian A, Kuchibhotla S, Koneru S, Strickman NE
Tex Heart Inst J 2022 Sep 1;49(5) doi: 10.14503/THIJ-22-7889. PMID: 36174575Free PMC Article
Luciani GB, De Rita F, Lucchese G, Barozzi L, Rossetti L, Faggian G, Mazzucco A
J Cardiovasc Med (Hagerstown) 2017 May;18(5):311-317. doi: 10.2459/JCM.0000000000000101. PMID: 25050529
Inoue Y, Watanabe M, Ando Y
J Stroke Cerebrovasc Dis 2014 May-Jun;23(5):1232-4. Epub 2013 Oct 15 doi: 10.1016/j.jstrokecerebrovasdis.2013.07.026. PMID: 24139408
Limongelli G, Pacileo G, Calabro' P, Rea A, Masarone D, Di Salvo G, D'Andrea A, Vatta M, Elliott PM, Calabro R
J Cardiovasc Med (Hagerstown) 2010 Mar;11(3):193-5. doi: 10.2459/JCM.0b013e32833134e9. PMID: 19996765
Fukuda T, Oku H, Nakamoto S, Mukobayashi M, Koike E
Circ J 2004 May;68(5):501-3. doi: 10.1253/circj.68.501. PMID: 15118296

Prognosis

Alifu A, Wang H, Su Y, Chen R
Medicine (Baltimore) 2022 Nov 25;101(47):e32070. doi: 10.1097/MD.0000000000032070. PMID: 36451474Free PMC Article
Sakai W, Asai H, Ebuoka N
Cardiol Young 2022 Nov;32(11):1864-1865. Epub 2022 Feb 28 doi: 10.1017/S1047951122000786. PMID: 35225204
Imai-Compton C, Elmi M, Manlhiot C, Floh AA, Golding F, Williams WG, McCrindle BW
Congenit Heart Dis 2010 Nov-Dec;5(6):532-6. doi: 10.1111/j.1747-0803.2010.00456.x. PMID: 21106011
Lopes LM, Rangel PI, Soraggi AM, Furlanetto BH, Furlanetto G
Arq Bras Cardiol 2001 Jun;76(6):511-6. doi: 10.1590/s0066-782x2001000600008. PMID: 11449296
Yeh T Jr, Connelly MS, Coles JG, Webb GD, McLaughlin PR, Freedom RM, Cerrito PB, Williams WG
J Thorac Cardiovasc Surg 1999 Jun;117(6):1190-203. doi: 10.1016/s0022-5223(99)70259-x. PMID: 10343272

Clinical prediction guides

Sun F, Fan M, Huang L, Ren W, Li D
J Clin Ultrasound 2018 May;46(4):265-268. Epub 2017 Sep 7 doi: 10.1002/jcu.22520. PMID: 28881023
Inoue Y, Watanabe M, Ando Y
J Stroke Cerebrovasc Dis 2014 May-Jun;23(5):1232-4. Epub 2013 Oct 15 doi: 10.1016/j.jstrokecerebrovasdis.2013.07.026. PMID: 24139408
Büchler JR, Carvalho HG, Ribeiro N
Jpn Heart J 1983 Jul;24(4):657-63. doi: 10.1536/ihj.24.657. PMID: 6645056
Steding G, Seidl W
Thorac Cardiovasc Surg 1981 Feb;29(1):1-16. doi: 10.1055/s-2007-1023435. PMID: 6164113
Cotrufo M, Nappi GA, d'Angelo A, Scardone M, de Vivo F
Int J Artif Organs 1980 May;3(3):181-8. PMID: 7429674

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