U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Abnormal vena cava morphology

MedGen UID:
1391621
•Concept ID:
C4476886
•
Anatomical Abnormality
Synonym: Abnormality of the vena cava
 
HPO: HP:0005345

Definition

An abnormality of the structure of the veins that return deoxygenated blood from the body into the heart, i.e., the superior vena cava and the inferior vena cava. [from HPO]

Professional guidelines

PubMed

Caval Thrombus Management: The Data, Where We Are, and How It Is Done.
Harrison B, Hao F, Koney N, McWilliams J, Moriarty JM
Tech Vasc Interv Radiol 2018 Jun;21(2):65-77. Epub 2018 Mar 9 doi: 10.1053/j.tvir.2018.03.002. PMID: 29784124
May-Thurner syndrome: diagnosis and management.
Brazeau NF, Harvey HB, Pinto EG, Deipolyi A, Hesketh RL, Oklu R
Vasa 2013 Mar;42(2):96-105. doi: 10.1024/0301-1526/a000252. PMID: 23485836
Prenatal diagnosis of ductus venosus agenesis and its association with cytogenetic/congenital anomalies.
Volpe P, Marasini M, Caruso G, Lituania M, Marzullo A, Volpe G, Gentile M
Prenat Diagn 2002 Nov;22(11):995-1000. doi: 10.1002/pd.456. PMID: 12424763

Recent clinical studies

Etiology

Coronary anomalies associated with ventricular septal defect.
Kobayashi M, Muneuchi J, Sugitani Y, Yamada A, Ezaki H, Watanabe M
Heart Vessels 2023 Feb;38(2):255-264. Epub 2022 Jul 26 doi: 10.1007/s00380-022-02144-6. PMID: 35882655
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
Hepatic Vein Incorporation Into the Azygos System in Heterotaxy and Interrupted Inferior Vena Cava.
Montesa C, Karamlou T, Ratnayaka K, Pophal SG, Ryan J, Nigro JJ
World J Pediatr Congenit Heart Surg 2019 May;10(3):330-337. doi: 10.1177/2150135119842869. PMID: 31084312
Ultrasound screening: Status of markers and efficacy of screening for structural abnormalities.
Rao R, Platt LD
Semin Perinatol 2016 Feb;40(1):67-78. Epub 2016 Jan 8 doi: 10.1053/j.semperi.2015.11.009. PMID: 26777687
Congenital portosystemic venous connections and other abdominal venous abnormalities in patients with polysplenia and functionally univentricular heart disease: a case series and literature review.
McElhinney DB, Marx GR, Newburger JW
Congenit Heart Dis 2011 Jan-Feb;6(1):28-40. doi: 10.1111/j.1747-0803.2010.00478.x. PMID: 21269410

Diagnosis

Prenatal diagnosis of hepatic interruption of the inferior vena cava with azygos/hemiazygos continuation without structural heart defects: A case series.
Babaoğlu K, Doğan Y, Başar EZ, Usta E
J Clin Ultrasound 2022 Jul;50(6):795-802. Epub 2022 Mar 30 doi: 10.1002/jcu.23209. PMID: 35355279
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
Fontan procedure: imaging of normal post-surgical anatomy and the spectrum of cardiac and extracardiac complications.
Navarro-Aguilar V, Flors L, Calvillo P, Merlos P, Buendía F, Igual B, Melero-Ferrer J, Soriano JR, Leiva-Salinas C
Clin Radiol 2015 Mar;70(3):295-303. Epub 2014 Nov 14 doi: 10.1016/j.crad.2014.10.005. PMID: 25468638
Congenital portosystemic venous connections and other abdominal venous abnormalities in patients with polysplenia and functionally univentricular heart disease: a case series and literature review.
McElhinney DB, Marx GR, Newburger JW
Congenit Heart Dis 2011 Jan-Feb;6(1):28-40. doi: 10.1111/j.1747-0803.2010.00478.x. PMID: 21269410
Coronary artery fistulae.
Luo L, Kebede S, Wu S, Stouffer GA
Am J Med Sci 2006 Aug;332(2):79-84. doi: 10.1097/00000441-200608000-00005. PMID: 16909054

Therapy

Non-invasive assessment of liver abnormalities in pediatric Fontan patients.
De Bruyne R, Vandekerckhove K, Van Overschelde H, Hendricx F, Vande Walle C, De Groote K, Panzer J, De Wolf D, Van Biervliet S, Bové T, François K
Eur J Pediatr 2022 Jan;181(1):159-169. Epub 2021 Jul 6 doi: 10.1007/s00431-021-04163-3. PMID: 34231051
Vascular Behçet's syndrome: an update.
Emmi G, Bettiol A, Silvestri E, Di Scala G, Becatti M, Fiorillo C, Prisco D
Intern Emerg Med 2019 Aug;14(5):645-652. Epub 2018 Nov 29 doi: 10.1007/s11739-018-1991-y. PMID: 30499073
Histopathological abnormalities in the central arteries and veins of Fontan subjects.
Hays BS, Baker M, Laib A, Tan W, Udholm S, Goldstein BH, Sanders SP, Opotowsky AR, Veldtman GR
Heart 2018 Feb;104(4):324-331. Epub 2017 Sep 29 doi: 10.1136/heartjnl-2017-311838. PMID: 28970278
Venoatrial shunt-assisted cavopulmonary anastomosis.
Kandakure PR, Dharmapuram AK, Kale SB, Babu V, Ramadoss N, Rao IM, Murthy KS
Asian Cardiovasc Thorac Ann 2010 Dec;18(6):569-73. doi: 10.1177/0218492310388459. PMID: 21149407
US of liver transplants: normal and abnormal.
Crossin JD, Muradali D, Wilson SR
Radiographics 2003 Sep-Oct;23(5):1093-114. doi: 10.1148/rg.235035031. PMID: 12975502

Prognosis

Early and Late Outcomes of the Warden and Modified Warden Procedure.
Griffeth EM, Dearani JA, Mathew J, Graham GC, Connolly HM, King KS, Schaff HV, Stephens EH
Ann Thorac Surg 2022 Nov;114(5):1723-1729. Epub 2022 Mar 26 doi: 10.1016/j.athoracsur.2022.03.032. PMID: 35351424Free PMC Article
Hepatic Vein Incorporation Into the Azygos System in Heterotaxy and Interrupted Inferior Vena Cava.
Montesa C, Karamlou T, Ratnayaka K, Pophal SG, Ryan J, Nigro JJ
World J Pediatr Congenit Heart Surg 2019 May;10(3):330-337. doi: 10.1177/2150135119842869. PMID: 31084312
Ultrasound screening: Status of markers and efficacy of screening for structural abnormalities.
Rao R, Platt LD
Semin Perinatol 2016 Feb;40(1):67-78. Epub 2016 Jan 8 doi: 10.1053/j.semperi.2015.11.009. PMID: 26777687
Left-sided and duplicate inferior vena cava: a case series and review.
Ang WC, Doyle T, Stringer MD
Clin Anat 2013 Nov;26(8):990-1001. Epub 2012 May 10 doi: 10.1002/ca.22090. PMID: 22576868
Coronary artery fistulae.
Luo L, Kebede S, Wu S, Stouffer GA
Am J Med Sci 2006 Aug;332(2):79-84. doi: 10.1097/00000441-200608000-00005. PMID: 16909054

Clinical prediction guides

Discovering a new part of the phenotypic spectrum of Coffin-Siris syndrome in a fetal cohort.
van der Sluijs PJ, Joosten M, Alby C, Attié-Bitach T, Gilmore K, Dubourg C, Fradin M, Wang T, Kurtz-Nelson EC, Ahlers KP, Arts P, Barnett CP, Ashfaq M, Baban A, van den Born M, Borrie S, Busa T, Byrne A, Carriero M, Cesario C, Chong K, Cueto-González AM, Dempsey JC, Diderich KEM, Doherty D, Farholt S, Gerkes EH, Gorokhova S, Govaerts LCP, Gregersen PA, Hickey SE, Lefebvre M, Mari F, Martinovic J, Northrup H, O'Leary M, Parbhoo K, Patrier S, Popp B, Santos-Simarro F, Stoltenburg C, Thauvin-Robinet C, Thompson E, Vulto-van Silfhout AT, Zahir FR, Scott HS, Earl RK, Eichler EE, Vora NL, Wilnai Y, Giordano JL, Wapner RJ, Rosenfeld JA, Haak MC, Santen GWE
Genet Med 2022 Aug;24(8):1753-1760. Epub 2022 May 18 doi: 10.1016/j.gim.2022.04.010. PMID: 35579625Free PMC Article
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
Angiopoietin-2 predicts morbidity in adults with Fontan physiology.
Shirali AS, Lluri G, Guihard PJ, Conrad MB, Kim H, Pawlikowska L, Boström KI, Iruela-Arispe ML, Aboulhosn JA
Sci Rep 2019 Dec 4;9(1):18328. doi: 10.1038/s41598-019-54776-w. PMID: 31797976Free PMC Article
Echocardiographic characteristics of the criss-cross heart.
Yang YL, Wang XF, Cheng TO, Xie MX, Lü Q, He L, Lu XF, Wang J, Li L, Anderson RH
Int J Cardiol 2010 Apr 15;140(2):133-7. Epub 2009 Sep 16 doi: 10.1016/j.ijcard.2009.08.039. PMID: 19762095
The somatic growth of autologous vessels in venous pathway after extracardiac total cavopulmonary connection.
Iwaki H, Masuda M, Shiokawa Y, Imoto Y, Nakano T, Sonoda H, Shiose A, Kado H
J Card Surg 2008 Sep-Oct;23(5):454-8. Epub 2008 May 7 doi: 10.1111/j.1540-8191.2008.00584.x. PMID: 18462342

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
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
Dilated cerebral venous system observed in growth-restricted fetuses.
Baron J, Mastrolia SA, Shelef I, Tirosh D, Daniel-Spiegel E, Hershkovitz R
J Matern Fetal Neonatal Med 2018 May;31(10):1369-1372. Epub 2017 Apr 20 doi: 10.1080/14767058.2017.1315097. PMID: 28372477
Left-sided and duplicate inferior vena cava: a case series and review.
Ang WC, Doyle T, Stringer MD
Clin Anat 2013 Nov;26(8):990-1001. Epub 2012 May 10 doi: 10.1002/ca.22090. PMID: 22576868
Endovascular stent-graft repair of major abdominal arteriovenous fistula: a systematic review.
Antoniou GA, Koutsias S, Karathanos C, Sfyroeras GS, Vretzakis G, Giannoukas AD
J Endovasc Ther 2009 Aug;16(4):514-23. doi: 10.1583/09-2725.1. PMID: 19702345

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • ClinVar
      Related medical variations
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...