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Discordant ventriculoarterial connection

MedGen UID:
760705
•Concept ID:
C3536741
•
Congenital Abnormality
Synonyms: Discordant Ventriculoarterial Connection; Discordant ventriculoarterial connection with concordant atrioventricular connection; TGA - Transposition of great arteries; TGV - Transposition of great vessels; Transposition of great arteries; Transposition of great vessels
SNOMED CT: Discordant ventriculoarterial connection (204296002); Transposition of great vessels (204296002); TGV - Transposition of great vessels (204296002); Transposition of great arteries (204296002); TGA - Transposition of great arteries (204296002); Discordant ventriculoarterial connection with concordant atrioventricular connection (26146002)

Definition

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

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDiscordant ventriculoarterial connection

Professional guidelines

PubMed

Prenatal diagnosis of tricuspid atresia: intrauterine course and outcome.
Berg C, Lachmann R, Kaiser C, Kozlowski P, Stressig R, Schneider M, Asfour B, Herberg U, Breuer J, Gembruch U, Geipel A
Ultrasound Obstet Gynecol 2010 Feb;35(2):183-90. doi: 10.1002/uog.7499. PMID: 20101636

Recent clinical studies

Etiology

Management of the Adult with Arterial Switch.
Breinholt JP, John S
Methodist Debakey Cardiovasc J 2019 Apr-Jun;15(2):133-137. doi: 10.14797/mdcj-15-2-133. PMID: 31384376Free PMC Article
Flow-sensitive four-dimensional velocity-encoded magnetic resonance imaging reveals abnormal blood flow patterns in the aorta and pulmonary trunk of patients with transposition.
Riesenkampff E, Nordmeyer S, Al-Wakeel N, Kropf S, Kutty S, Berger F, Kuehne T
Cardiol Young 2014 Feb;24(1):47-53. Epub 2013 Jan 18 doi: 10.1017/S1047951112002193. PMID: 23327710
Morphologic spectrum of ventriculoarterial connection in hearts with double inlet left ventricle: implications for surgical procedures.
Uemura H, Ho SY, Adachi I, Yagihara T
Ann Thorac Surg 2008 Oct;86(4):1321-7. doi: 10.1016/j.athoracsur.2008.06.050. PMID: 18805185
Subaortic obstruction and the Fontan operation.
Freedom RM
Ann Thorac Surg 1998 Aug;66(2):649-52. doi: 10.1016/s0003-4975(98)00619-5. PMID: 9725446
Death and other events after cardiac repair in discordant atrioventricular connection.
McGrath LB, Kirklin JW, Blackstone EH, Pacifico AD, Kirklin JK, Bargeron LM Jr
J Thorac Cardiovasc Surg 1985 Nov;90(5):711-28. PMID: 4058044

Diagnosis

Mechanism responsible for D-transposition of the great arteries: Is this part of the spectrum of right isomerism?
Nakajima Y
Congenit Anom (Kyoto) 2016 Sep;56(5):196-202. doi: 10.1111/cga.12176. PMID: 27329052
Flow-sensitive four-dimensional velocity-encoded magnetic resonance imaging reveals abnormal blood flow patterns in the aorta and pulmonary trunk of patients with transposition.
Riesenkampff E, Nordmeyer S, Al-Wakeel N, Kropf S, Kutty S, Berger F, Kuehne T
Cardiol Young 2014 Feb;24(1):47-53. Epub 2013 Jan 18 doi: 10.1017/S1047951112002193. PMID: 23327710
Prenatal diagnosis of tricuspid atresia: intrauterine course and outcome.
Berg C, Lachmann R, Kaiser C, Kozlowski P, Stressig R, Schneider M, Asfour B, Herberg U, Breuer J, Gembruch U, Geipel A
Ultrasound Obstet Gynecol 2010 Feb;35(2):183-90. doi: 10.1002/uog.7499. PMID: 20101636
Is banding of the pulmonary trunk obsolete for infants with tricuspid atresia and double inlet ventricle with a discordant ventriculoarterial connection? Role of aortic arch obstruction and subaortic stenosis.
Franklin RC, Sullivan ID, Anderson RH, Shinebourne EA, Deanfield JE
J Am Coll Cardiol 1990 Nov;16(6):1455-64. doi: 10.1016/0735-1097(90)90392-3. PMID: 1699983
Diagnosis and significance of atrial isomerism.
Sapire DW, Ho SY, Anderson RH, Rigby ML
Am J Cardiol 1986 Aug 1;58(3):342-6. doi: 10.1016/0002-9149(86)90074-3. PMID: 3739926

Therapy

Management of the Adult with Arterial Switch.
Breinholt JP, John S
Methodist Debakey Cardiovasc J 2019 Apr-Jun;15(2):133-137. doi: 10.14797/mdcj-15-2-133. PMID: 31384376Free PMC Article
Is banding of the pulmonary trunk obsolete for infants with tricuspid atresia and double inlet ventricle with a discordant ventriculoarterial connection? Role of aortic arch obstruction and subaortic stenosis.
Franklin RC, Sullivan ID, Anderson RH, Shinebourne EA, Deanfield JE
J Am Coll Cardiol 1990 Nov;16(6):1455-64. doi: 10.1016/0735-1097(90)90392-3. PMID: 1699983

Prognosis

Prenatal diagnosis of tricuspid atresia: intrauterine course and outcome.
Berg C, Lachmann R, Kaiser C, Kozlowski P, Stressig R, Schneider M, Asfour B, Herberg U, Breuer J, Gembruch U, Geipel A
Ultrasound Obstet Gynecol 2010 Feb;35(2):183-90. doi: 10.1002/uog.7499. PMID: 20101636
Morphologic spectrum of ventriculoarterial connection in hearts with double inlet left ventricle: implications for surgical procedures.
Uemura H, Ho SY, Adachi I, Yagihara T
Ann Thorac Surg 2008 Oct;86(4):1321-7. doi: 10.1016/j.athoracsur.2008.06.050. PMID: 18805185
Bidirectional superior cavopulmonary anastomosis: how young is too young?
Slavik Z, Lamb RK, Webber SA, Devlin AM, Keeton BR, Monro JL, Salmon AP
Heart 1996 Jan;75(1):78-82. doi: 10.1136/hrt.75.1.78. PMID: 8624878Free PMC Article
Direct relief of subaortic obstruction in patients with univentricular atrioventricular connection and discordant ventriculoarterial connection: intermediate results.
Ross DB, Cheung HC, Lincoln C
Semin Thorac Cardiovasc Surg 1994 Jan;6(1):33-8. PMID: 8167170
Atresia of left atrioventricular orifice. Anatomical investigation in 62 cases.
Thiene G, Daliento L, Frescura C, De Tommasi M, Macartney FJ, Anderson RH
Br Heart J 1981 Apr;45(4):393-401. doi: 10.1136/hrt.45.4.393. PMID: 7225254Free PMC Article

Clinical prediction guides

Flow-sensitive four-dimensional velocity-encoded magnetic resonance imaging reveals abnormal blood flow patterns in the aorta and pulmonary trunk of patients with transposition.
Riesenkampff E, Nordmeyer S, Al-Wakeel N, Kropf S, Kutty S, Berger F, Kuehne T
Cardiol Young 2014 Feb;24(1):47-53. Epub 2013 Jan 18 doi: 10.1017/S1047951112002193. PMID: 23327710
Atresia of left atrioventricular orifice. Anatomical investigation in 62 cases.
Thiene G, Daliento L, Frescura C, De Tommasi M, Macartney FJ, Anderson RH
Br Heart J 1981 Apr;45(4):393-401. doi: 10.1136/hrt.45.4.393. PMID: 7225254Free PMC Article

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