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Double inlet right ventricle

MedGen UID:
488861
Concept ID:
C0344621
Congenital Abnormality
Synonym: DIRV - Double inlet right ventricle
SNOMED CT: DIRV - Double inlet right ventricle (253282005); Double inlet right ventricle (253282005)
 
HPO: HP:0011556

Definition

The condition in which both atria are joined to the right ventricle each by its own atrioventricular valve. Usually, the left ventricle is hypoplastic. [from HPO]

Conditions with this feature

Heterotaxy, visceral, 12, autosomal
MedGen UID:
1803695
Concept ID:
C5676898
Congenital Abnormality
Visceral heterotaxy-12 (HTX12) is an embryonic developmental disorder characterized by defects in the asymmetric positioning of visceral organs across the left-right axis, known as laterality defects. The phenotype is highly variable, ranging from complete organ reversal (situs inversus totalis) to selective misarrangement of organs (situs ambiguus) such as the liver, spleen, and pancreas. The disorder is often associated with dextrocardia or variable complex congenital heart defects. Early death may occur in the most severe cases (summary by Szenker-Ravi et al., 2022). For a discussion of the genetic heterogeneity of visceral heterotaxy, see HTX1 (306955).

Professional guidelines

PubMed

Brida M, Diller GP, Gatzoulis MA
Circulation 2018 Jan 30;137(5):508-518. doi: 10.1161/CIRCULATIONAHA.117.031544. PMID: 29378757

Recent clinical studies

Etiology

Sinha M, Pandey NN, Sharma A, Kumar S
J Card Surg 2020 Nov;35(11):2979-2986. Epub 2020 Aug 16 doi: 10.1111/jocs.14960. PMID: 33111443
Brida M, Diller GP, Gatzoulis MA
Circulation 2018 Jan 30;137(5):508-518. doi: 10.1161/CIRCULATIONAHA.117.031544. PMID: 29378757
Zhu ZQ, Hong HF, Chen HW, Zhang HB, Lu ZH, Zheng JH, Shi GC, Liu JF
Ann Thorac Surg 2015 Jan;99(1):156-61. Epub 2014 Nov 13 doi: 10.1016/j.athoracsur.2014.08.021. PMID: 25440283
Kawahira Y, Uemura H, Yoshikawa Y, Yagihara T
Eur J Cardiothorac Surg 2001 Aug;20(2):228-32. doi: 10.1016/s1010-7940(01)00805-3. PMID: 11463536
Wong ML, Sim EK, Goh JJ, Quek SC, Wong JC, Yip WC, Lee CN
Ann Acad Med Singap 1999 Mar;28(2):237-40. PMID: 10497674

Diagnosis

Sinha M, Pandey NN, Sharma A, Kumar S
J Card Surg 2020 Nov;35(11):2979-2986. Epub 2020 Aug 16 doi: 10.1111/jocs.14960. PMID: 33111443
Kim TH, Yoo SJ, Ho SY, Anderson RH
Cardiol Young 2000 Nov;10(6):567-73. doi: 10.1017/s1047951100008830. PMID: 11117388
Wang JK, Lue HC, Wu MH, Chiu IS, Hung CR
Am J Cardiol 1993 Jul 1;72(1):85-9. doi: 10.1016/0002-9149(93)90224-z. PMID: 8517435
Shiraishi H, Silverman NH
J Am Coll Cardiol 1990 May;15(6):1401-8. doi: 10.1016/s0735-1097(10)80031-2. PMID: 2329242
Freedom RM, Bini M, Rowe RD
Eur J Cardiol 1978 Jun;7(4):263-81. PMID: 567583

Therapy

Mostefa-Kara M, Houyel L, Bonnet D
Orphanet J Rare Dis 2018 Jul 18;13(1):118. doi: 10.1186/s13023-018-0861-z. PMID: 30021599Free PMC Article
Brida M, Diller GP, Gatzoulis MA
Circulation 2018 Jan 30;137(5):508-518. doi: 10.1161/CIRCULATIONAHA.117.031544. PMID: 29378757
Pontailler M, Gaudin R, Lenoir M, Haydar A, Kraiche D, Bonnet D, Vouhé P, Raisky O
Eur J Cardiothorac Surg 2017 May 1;51(5):1003-1008. doi: 10.1093/ejcts/ezx021. PMID: 28329111

Prognosis

Brida M, Diller GP, Gatzoulis MA
Circulation 2018 Jan 30;137(5):508-518. doi: 10.1161/CIRCULATIONAHA.117.031544. PMID: 29378757
Matsuura K, Akizuki S, Nakamura N, Ishibashi-Ueda H, Moriyama M
South Med J 2007 Feb;100(2):218-21. doi: 10.1097/01.smj.0000242349.55168.a7. PMID: 17330698
Margossian RE, Solowiejczyk D, Bourlon F, Apfel H, Gersony WM, Hordof AJ, Quaegebeur J
J Thorac Cardiovasc Surg 2002 Sep;124(3):442-7. doi: 10.1067/mtc.2002.122299. PMID: 12202859
Wong ML, Sim EK, Goh JJ, Quek SC, Wong JC, Yip WC, Lee CN
Ann Acad Med Singap 1999 Mar;28(2):237-40. PMID: 10497674
Matsuda H, Kawashima Y, Kishimoto H, Hirose H, Nakano S, Kato H, Taniguchi K, Nishigaki K, Sano T, Ogawa M
Circulation 1987 Sep;76(3 Pt 2):III45-52. PMID: 3621542

Clinical prediction guides

Sinha M, Pandey NN, Sharma A, Kumar S
J Card Surg 2020 Nov;35(11):2979-2986. Epub 2020 Aug 16 doi: 10.1111/jocs.14960. PMID: 33111443
Spadotto V, Frescura C, Ho SY, Thiene G
Cardiovasc Pathol 2017 Jan-Feb;26:39-44. Epub 2016 Sep 25 doi: 10.1016/j.carpath.2016.09.003. PMID: 27866077

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