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Absent right superior vena cava

MedGen UID:
488865
Concept ID:
C0344656
Congenital Abnormality
SNOMED CT: Absent right superior vena cava (253308005)
 
HPO: HP:0011666

Definition

Absence of the right superior vena cava (RSVC). An absent RSVC is always associated with a persistent left superior vena cava (PLSVC). During normal fetal development, the left-sided anterior venous cardinal system regresses, leaving the coronary sinus (CS) and the ligament of Marshall. Failure of the closure of the left anterior cardinal vein results in PLSVC. In general, PLSVC is associated with the right superior vena cava (RSVC) and drains into the RA via a dilated CS. When developmental arrest occurs at an earlier stage, the CS is absent and the PLSVC drains into the left atrium. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAbsent right superior vena cava

Recent clinical studies

Etiology

Araki H, Sekino M, Iwasaki N, Suzumura M, Egashira T, Yano R, Matsumoto S, Higashijima U, Sugimoto T, Yamanashi H, Hara T
J Artif Organs 2022 Jun;25(2):170-173. Epub 2021 Aug 16 doi: 10.1007/s10047-021-01290-4. PMID: 34401951Free PMC Article
Ichikawa T, Hara T, Koizumi J, Nakamura N, Nagafuji Y, Furuya H, Mikami T, Kuroki T, Hashimoto J
Jpn J Radiol 2020 Nov;38(11):1046-1051. Epub 2020 Jul 14 doi: 10.1007/s11604-020-01013-4. PMID: 32666181
Rizkallah J, Burgess J, Kuriachan V
BMC Res Notes 2014 Jul 21;7:462. doi: 10.1186/1756-0500-7-462. PMID: 25047923Free PMC Article
Moorthy N, Kapoor A, Kumar S
Indian Heart J 2013 Sep-Oct;65(5):603-6. Epub 2013 Aug 26 doi: 10.1016/j.ihj.2013.08.024. PMID: 24206885Free PMC Article
Mazzucco A, Bortolotti U, Stellin G, Gallucci V
J Card Surg 1990 Jun;5(2):122-33. doi: 10.1111/j.1540-8191.1990.tb00749.x. PMID: 2133830

Diagnosis

Smolarek D, Jankowska H, Dorniak K, Hellmann M
J Cardiothorac Surg 2024 Apr 4;19(1):175. doi: 10.1186/s13019-024-02709-8. PMID: 38575998Free PMC Article
Joshi S, Singh A
Ann Card Anaesth 2023 Oct-Dec;26(4):433-434. doi: 10.4103/aca.aca_91_23. PMID: 37861579Free PMC Article
Misra S, Mohanty S, Sathia S, Mahapatra RP, Tapuria P
Ann Card Anaesth 2021 Jul-Sep;24(3):358-361. doi: 10.4103/aca.ACA_198_20. PMID: 34269268Free PMC Article
Patel Y, Gupta R
Methodist Debakey Cardiovasc J 2018 Jul-Sep;14(3):232-235. doi: 10.14797/mdcj-14-3-232. PMID: 30410655Free PMC Article
Mazzucco A, Bortolotti U, Stellin G, Gallucci V
J Card Surg 1990 Jun;5(2):122-33. doi: 10.1111/j.1540-8191.1990.tb00749.x. PMID: 2133830

Therapy

Kale RD, Sarwar MF, Sopchak A
J Cardiothorac Vasc Anesth 2019 Jan;33(1):157-161. Epub 2018 Feb 2 doi: 10.1053/j.jvca.2018.01.054. PMID: 29525189
Saranteas T, Mandila C, Poularas J, Papanikolaou J, Patriankos A, Karakitsos D, Karabinis A
Eur J Echocardiogr 2009 May;10(3):452-5. Epub 2009 Feb 27 doi: 10.1093/ejechocard/jen334. PMID: 19252187
Kilickap M, Altin T, Akyurek O, Karaoguz R, Akgun G, Guldal M
Can J Cardiol 2005 Nov;21(13):1221-3. PMID: 16308601

Prognosis

Patel Y, Gupta R
Methodist Debakey Cardiovasc J 2018 Jul-Sep;14(3):232-235. doi: 10.14797/mdcj-14-3-232. PMID: 30410655Free PMC Article
Doksöz Ö, Güven B, Yozgat Y, Özdemir R, Meşe T, Tavlı V, Alayunt EA
Congenit Heart Dis 2014 Mar-Apr;9(2):E37-40. Epub 2013 Apr 22 doi: 10.1111/chd.12065. PMID: 23602062
Moorthy N, Kapoor A, Kumar S
Indian Heart J 2013 Sep-Oct;65(5):603-6. Epub 2013 Aug 26 doi: 10.1016/j.ihj.2013.08.024. PMID: 24206885Free PMC Article
Vodiskar J, Clur SA, Hruda J, Bokenkamp R, Hazekamp MG
Eur J Cardiothorac Surg 2010 Jun;37(6):1259-63. Epub 2010 Feb 12 doi: 10.1016/j.ejcts.2009.12.041. PMID: 20153212
Mooney DP, Snyder CL, Holder TM
J Pediatr Surg 1993 Dec;28(12):1633-4. doi: 10.1016/0022-3468(93)90125-5. PMID: 8301517

Clinical prediction guides

Araki H, Sekino M, Iwasaki N, Suzumura M, Egashira T, Yano R, Matsumoto S, Higashijima U, Sugimoto T, Yamanashi H, Hara T
J Artif Organs 2022 Jun;25(2):170-173. Epub 2021 Aug 16 doi: 10.1007/s10047-021-01290-4. PMID: 34401951Free PMC Article
Doksöz Ö, Güven B, Yozgat Y, Özdemir R, Meşe T, Tavlı V, Alayunt EA
Congenit Heart Dis 2014 Mar-Apr;9(2):E37-40. Epub 2013 Apr 22 doi: 10.1111/chd.12065. PMID: 23602062
Mooney DP, Snyder CL, Holder TM
J Pediatr Surg 1993 Dec;28(12):1633-4. doi: 10.1016/0022-3468(93)90125-5. PMID: 8301517

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