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Congenital Vena Cava Abnormality

MedGen UID:
476118
•Concept ID:
C3274485
•
Disease or Syndrome
Synonym: Congenital Abnormality of Vena Cava

Definition

An abnormality of the superior or inferior vena cava that is present at birth. Representative examples include persistent left superior vena cava, absence of infrarenal inferior vena cava, or absence of the entire inferior vena cava. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenital Vena Cava Abnormality

Professional guidelines

PubMed

Outcomes following prenatal diagnosis of isolated persistent left superior vena cava.
Durand I, Hazelzet T, Gillibert A, Parrod C, David N, El Youssef F, Brehin AC, Barre E
Arch Cardiovasc Dis 2022 Jun-Jul;115(6-7):335-347. Epub 2022 May 14 doi: 10.1016/j.acvd.2022.03.005. PMID: 35660361
The frequency and efficacy of genetic testing in individuals with scimitar syndrome.
Fick TA, Scott DA, Lupo PJ, Weigand J, Morris SA
Cardiol Young 2022 Apr;32(4):550-557. Epub 2021 Jul 2 doi: 10.1017/S1047951121002535. PMID: 34210367Free PMC Article
Persistent Left Superior Vena Cava: Why is Prenatal Diagnosis Important?
Keleş A, Yılmaz O, Dağdeviren G, Çelik ÖY, Yücel A, Şahin D
Fetal Pediatr Pathol 2022 Aug;41(4):592-602. Epub 2021 Jun 9 doi: 10.1080/15513815.2021.1933662. PMID: 34106033

Recent clinical studies

Etiology

Inferior vena cava thrombosis-rope ladder sign.
Meyer S, Poryo M
Wien Med Wochenschr 2023 May;173(7-8):159-160. Epub 2021 Oct 6 doi: 10.1007/s10354-021-00886-y. PMID: 34613517Free PMC Article
Single left superior vena cava: antenatal diagnosis, associated anomalies and outcomes.
Lopes KRM, Bartsota M, Doughty V, Carvalho JS
Ultrasound Obstet Gynecol 2022 Nov;60(5):640-645. doi: 10.1002/uog.24966. PMID: 35656845Free PMC Article
Clinical implications of congenital interruption of inferior vena cava.
Chen SJ, Wu MH, Wang JK
J Formos Med Assoc 2022 Oct;121(10):1938-1944. Epub 2022 Feb 5 doi: 10.1016/j.jfma.2022.01.021. PMID: 35135704
Anomalies of Inferior Vena Cava: Implications and Considerations in Retroperitoneal Surgical Procedures.
Nigro B, Ayarragaray JEF
Ann Vasc Surg 2022 Feb;79:372-379. Epub 2021 Oct 10 doi: 10.1016/j.avsg.2021.07.029. PMID: 34644626
Diagnosis of fetal non-chromosomal abnormalities on routine ultrasound examination at 11-13 weeks' gestation.
Syngelaki A, Hammami A, Bower S, Zidere V, Akolekar R, Nicolaides KH
Ultrasound Obstet Gynecol 2019 Oct;54(4):468-476. doi: 10.1002/uog.20844. PMID: 31408229

Diagnosis

Outcomes following prenatal diagnosis of isolated persistent left superior vena cava.
Durand I, Hazelzet T, Gillibert A, Parrod C, David N, El Youssef F, Brehin AC, Barre E
Arch Cardiovasc Dis 2022 Jun-Jul;115(6-7):335-347. Epub 2022 May 14 doi: 10.1016/j.acvd.2022.03.005. PMID: 35660361
Single left superior vena cava: antenatal diagnosis, associated anomalies and outcomes.
Lopes KRM, Bartsota M, Doughty V, Carvalho JS
Ultrasound Obstet Gynecol 2022 Nov;60(5):640-645. doi: 10.1002/uog.24966. PMID: 35656845Free PMC Article
Various congenital anomalies of the inferior vena cava: review of cross-sectional imaging findings and report of a new variant.
Kim SS, Shin HC, Hwang JA, Jou SS, Lee WH, Choi SY, Park CH
Abdom Radiol (NY) 2018 Aug;43(8):2130-2149. doi: 10.1007/s00261-017-1430-y. PMID: 29264715
Persistent Left Superior Vena Cava in Fetuses: An Autopsy Series.
Ramakrishnan D, Chidambarathanu S, Murli L, Micheal J, Jagadeesh S, Suresh I, Seshadri S
Fetal Pediatr Pathol 2017 Aug;36(4):304-310. Epub 2017 Jun 1 doi: 10.1080/15513815.2017.1324546. PMID: 28569558
Congenital anomalies of the aorta and vena cava: 16-detector-row CT imaging findings.
Onbaş O, Kantarci M, Koplay M, Olgun H, Alper F, Aydinli B, Zirek H, Ceviz N
Diagn Interv Radiol 2008 Sep;14(3):163-71. PMID: 18814140

Therapy

Vena Cava Thrombosis after Congenital Diaphragmatic Hernia Repair: Multivariate Analysis of Potential Risk Factors.
Kiblawi R, Zoeller C, Pirr S, Hofmann AD, Ure B, Dingemann J
Eur J Pediatr Surg 2022 Feb;32(1):91-97. Epub 2021 Dec 10 doi: 10.1055/s-0041-1740462. PMID: 34891190
Anomalies of Inferior Vena Cava: Implications and Considerations in Retroperitoneal Surgical Procedures.
Nigro B, Ayarragaray JEF
Ann Vasc Surg 2022 Feb;79:372-379. Epub 2021 Oct 10 doi: 10.1016/j.avsg.2021.07.029. PMID: 34644626
Pediatric superior vena cava syndrome: An evidence-based systematic review of the literature.
Nossair F, Schoettler P, Starr J, Chan AKC, Kirov I, Paes B, Mahajerin A
Pediatr Blood Cancer 2018 Sep;65(9):e27225. Epub 2018 May 21 doi: 10.1002/pbc.27225. PMID: 29781569
Inferior Vena Cava Thrombosis.
Alkhouli M, Morad M, Narins CR, Raza F, Bashir R
JACC Cardiovasc Interv 2016 Apr 11;9(7):629-43. Epub 2016 Mar 4 doi: 10.1016/j.jcin.2015.12.268. PMID: 26952909
Transcatheter Fontan takedown.
Hallbergson A, Mascio CE, Rome JJ
Catheter Cardiovasc Interv 2015 Nov;86(5):849-54. Epub 2015 May 6 doi: 10.1002/ccd.25963. PMID: 25945427

Prognosis

Anomalies of Inferior Vena Cava: Implications and Considerations in Retroperitoneal Surgical Procedures.
Nigro B, Ayarragaray JEF
Ann Vasc Surg 2022 Feb;79:372-379. Epub 2021 Oct 10 doi: 10.1016/j.avsg.2021.07.029. PMID: 34644626
Surgical management of the scimitar syndrome.
Chowdhury UK, Anderson RH, Sankhyan LK, George N, Pandey NN, Chauhan AS, Arora Y, Goja S
J Card Surg 2021 Oct;36(10):3770-3795. Epub 2021 Aug 16 doi: 10.1111/jocs.15857. PMID: 34396590
Diagnosis of major heart defects by routine first-trimester ultrasound examination: association with increased nuchal translucency, tricuspid regurgitation and abnormal flow in ductus venosus.
Minnella GP, Crupano FM, Syngelaki A, Zidere V, Akolekar R, Nicolaides KH
Ultrasound Obstet Gynecol 2020 May;55(5):637-644. doi: 10.1002/uog.21956. PMID: 31875326
Left superior vena cava: cross-sectional imaging overview.
Batouty NM, Sobh DM, Gadelhak B, Sobh HM, Mahmoud W, Tawfik AM
Radiol Med 2020 Mar;125(3):237-246. Epub 2019 Dec 10 doi: 10.1007/s11547-019-01114-9. PMID: 31823296
Eponym. Scimitar syndrome.
Midyat L, Demir E, Aşkin M, Gülen F, Ulger Z, Tanaç R, Bayraktaroğlu S
Eur J Pediatr 2010 Oct;169(10):1171-7. Epub 2010 Mar 12 doi: 10.1007/s00431-010-1152-4. PMID: 20225123

Clinical prediction guides

Prevalence of Inferior Vena Cava Anomalies and Their Significance and Impact in Clinical Practice.
Kim H, Labropoulos N, Blake AM, Desai K
Eur J Vasc Endovasc Surg 2022 Oct;64(4):388-394. Epub 2022 Jun 6 doi: 10.1016/j.ejvs.2022.05.045. PMID: 35671937
Clinical significance of prenatally diagnosed persistent left superior vena cava.
Esin D, Aslan Çetin B, Şenol G, Selçuki NFT, Gedik Özköse Z, Acar Z, Yüksel MA
J Gynecol Obstet Hum Reprod 2022 Apr;51(4):102332. Epub 2022 Feb 2 doi: 10.1016/j.jogoh.2022.102332. PMID: 35123124
Anomalies of Inferior Vena Cava: Implications and Considerations in Retroperitoneal Surgical Procedures.
Nigro B, Ayarragaray JEF
Ann Vasc Surg 2022 Feb;79:372-379. Epub 2021 Oct 10 doi: 10.1016/j.avsg.2021.07.029. PMID: 34644626
Diagnosis of major heart defects by routine first-trimester ultrasound examination: association with increased nuchal translucency, tricuspid regurgitation and abnormal flow in ductus venosus.
Minnella GP, Crupano FM, Syngelaki A, Zidere V, Akolekar R, Nicolaides KH
Ultrasound Obstet Gynecol 2020 May;55(5):637-644. doi: 10.1002/uog.21956. PMID: 31875326
Pediatric superior vena cava syndrome: An evidence-based systematic review of the literature.
Nossair F, Schoettler P, Starr J, Chan AKC, Kirov I, Paes B, Mahajerin A
Pediatr Blood Cancer 2018 Sep;65(9):e27225. Epub 2018 May 21 doi: 10.1002/pbc.27225. PMID: 29781569

Recent systematic reviews

Systematic review of electrophysiology procedures in patients with obstruction of the inferior vena cava.
Al-Sinan A, Chan KH, Young GD, Martin A, Sepahpour A, Sy RW
J Cardiovasc Electrophysiol 2022 Jun;33(6):1300-1311. Epub 2022 Apr 30 doi: 10.1111/jce.15505. PMID: 35441755Free PMC Article
Clinical and procedural implications of congenital vena cava anomalies in adults: A systematic review.
Shafi I, Hassan AAI, Akers KG, Bashir R, Alkhouli M, Weinberger JJ, Abidov A
Int J Cardiol 2020 Sep 15;315:29-35. Epub 2020 May 8 doi: 10.1016/j.ijcard.2020.05.017. PMID: 32434672
Duplication and transposition of inferior vena cava: A meta-analysis of prevalence.
Hostiuc S, Minoiu C, Negoi I, Rusu MC, Hostiuc M
J Vasc Surg Venous Lymphat Disord 2019 Sep;7(5):742-755. Epub 2019 May 5 doi: 10.1016/j.jvsv.2019.01.063. PMID: 31068277
Pediatric superior vena cava syndrome: An evidence-based systematic review of the literature.
Nossair F, Schoettler P, Starr J, Chan AKC, Kirov I, Paes B, Mahajerin A
Pediatr Blood Cancer 2018 Sep;65(9):e27225. Epub 2018 May 21 doi: 10.1002/pbc.27225. PMID: 29781569
Systematic review and meta-analysis of persistent left superior vena cava on prenatal ultrasound: associated anomalies, diagnostic accuracy and postnatal outcome.
Gustapane S, Leombroni M, Khalil A, Giacci F, Marrone L, Bascietto F, Rizzo G, Acharya G, Liberati M, D'Antonio F
Ultrasound Obstet Gynecol 2016 Dec;48(6):701-708. Epub 2016 Nov 1 doi: 10.1002/uog.15914. PMID: 26970258

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