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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.
See: This record
Not genetically inherited (Orphanet)
 
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).
See: Condition Record
Pancreatic hypoplasia-diabetes-congenital heart disease syndrome

Professional guidelines

PubMed

Double outlet left ventricle. Morphology, cineangiocardiographic diagnosis and surgical treatment.
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

Double outlet ventricles: review of anatomic and imaging characteristics.
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
Bi-ventricular repair of double outlet left ventricle: Experience and review of the literature.
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
Current management of double-outlet left ventricle: towards biventricular repair in infancy.
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
Characteristics and outcomes of double outlet left ventricle.
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
Double-outlet left ventricle. Echocardiographic diagnosis.
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

Double outlet ventricles: review of anatomic and imaging characteristics.
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
Biventricular repair of double-outlet left ventricle by handmade trileaflet-valved conduit: A case report.
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
Absent pulmonary valve with double-outlet left ventricle: a case report.
Sakai W, Asai H, Ebuoka N
Cardiol Young 2022 Nov;32(11):1864-1865. Epub 2022 Feb 28 doi: 10.1017/S1047951122000786. PMID: 35225204
Congenital Heart Surgery Nomenclature and Database Project: double outlet left ventricle.
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
Double outlet left ventricle.
Anderson R, Galbraith R, Gibson R, Miller G
Br Heart J 1974 Jun;36(6):554-8. doi: 10.1136/hrt.36.6.554. PMID: 4851434Free PMC Article

Therapy

A Special Clinical Scenario for Transcatheter Aortic Valve Replacement: "Iatrogenic Double-Outlet" Left Ventricle.
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
Current management of double-outlet left ventricle: towards biventricular repair in infancy.
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
Twenty-five years' experience of modified Lecompte procedure for the anomalies of ventriculoarterial connection with ventricular septal defect and pulmonary stenosis.
Lim HG, Kim WH, Lee JR, Kim YJ
J Thorac Cardiovasc Surg 2014 Sep;148(3):825-31. Epub 2014 Jun 13 doi: 10.1016/j.jtcvs.2014.05.074. PMID: 25037623
Brain infarction with a predilection for cerebellum in a patient with double-outlet left ventricle and vascular malformations.
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
Right ventricular hypertrabeculation associated with double-outlet left ventricle: exaggeration of a normal pattern or right ventricular cardiomyopathy?
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

Prognosis

Biventricular repair of double-outlet left ventricle by handmade trileaflet-valved conduit: A case report.
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
Absent pulmonary valve with double-outlet left ventricle: a case report.
Sakai W, Asai H, Ebuoka N
Cardiol Young 2022 Nov;32(11):1864-1865. Epub 2022 Feb 28 doi: 10.1017/S1047951122000786. PMID: 35225204
Characteristics and outcomes of double outlet left ventricle.
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
Double-outlet left ventricle. Echocardiographic diagnosis.
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
Atrioventricular discordance: results of repair in 127 patients.
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

Double-outlet left ventricle with a bicuspid pulmonary valve and aortic coarctation.
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
Brain infarction with a predilection for cerebellum in a patient with double-outlet left ventricle and vascular malformations.
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
Contribution to the development of the heart, Part II: Morphogenesis of congenital heart diseases.
Steding G, Seidl W
Thorac Cardiovasc Surg 1981 Feb;29(1):1-16. doi: 10.1055/s-2007-1023435. PMID: 6164113
Prosthetic creation of a double outlet left ventricle: clinical experience of five cases.
Cotrufo M, Nappi GA, d'Angelo A, Scardone M, de Vivo F
Int J Artif Organs 1980 May;3(3):181-8. PMID: 7429674
Cardiac connections: the segmental approach to radiologic diagnosis in congenital heart disease.
Brandt PW, Calder AL
Curr Probl Diagn Radiol 1977 May-Jun;7(3):1-35. doi: 10.1016/s0363-0188(77)80006-6. PMID: 872614

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