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Abnormal vena cava physiology

MedGen UID:
1377349
•Concept ID:
C4476885
•
Finding
HPO: HP:0030970

Definition

An abnormality of the function 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]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAbnormal vena cava physiology

Professional guidelines

PubMed

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
Oncologic Emergencies: Recognition and Initial Management.
Higdon ML, Atkinson CJ, Lawrence KV
Am Fam Physician 2018 Jun 1;97(11):741-748. PMID: 30215936
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

Recent clinical studies

Etiology

Fetal Hemodynamics, Early Survival, and Neurodevelopment in Patients With Cyanotic Congenital Heart Disease.
Lee FT, Sun L, van Amerom JFP, Portnoy S, Marini D, Saini A, Milligan N, Lim JM, Saini B, Selvanathan T, Kazazian V, Sananes R, Jaeggi E, Kingdom JC, Macgowan CK, Ly L, Chau V, Miller SP, Seed M
J Am Coll Cardiol 2024 Apr 2;83(13):1225-1239. Epub 2024 Mar 25 doi: 10.1016/j.jacc.2024.02.005. PMID: 38538202
Cardiovascular Manifestations of Turner Syndrome: Phenotypic Differences Between Karyotype Subtypes.
Birjiniuk A, Weisman AG, Laternser C, Camarda J, Brickman WJ, Habiby R, Patel SR
Pediatr Cardiol 2024 Oct;45(7):1407-1414. Epub 2023 May 5 doi: 10.1007/s00246-023-03159-0. PMID: 37147524
IVC measurement for the noninvasive evaluation of central venous pressure.
Ruge M, Marhefka GD
J Echocardiogr 2022 Sep;20(3):133-143. Epub 2022 Apr 1 doi: 10.1007/s12574-022-00569-6. PMID: 35362870
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
The hemi-Fontan operation.
Jacobs ML, Pourmoghadam KK
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2003;6:90-7. doi: 10.1053/pcsu.2003.50007. PMID: 12740775

Diagnosis

Ultrasound Imaging of the Superior Vena Cava: A State-of-the-Art Review.
Fadel BM, Kazzi B, Mohty D
J Am Soc Echocardiogr 2023 May;36(5):447-463. Epub 2023 Feb 7 doi: 10.1016/j.echo.2023.01.017. PMID: 36754099
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
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
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
Aortocaval fistula: current management strategies.
Brightwell RE, Pegna V, Boyne N
ANZ J Surg 2013 Jan;83(1-2):31-5. Epub 2012 Oct 16 doi: 10.1111/j.1445-2197.2012.06294.x. PMID: 23072669

Therapy

Transcaval Access for Large Bore Devices.
Eng MH, Villablanca P, Frisoli T, Greenbaum AB, O'Neill WW
Curr Cardiol Rep 2019 Oct 31;21(11):134. doi: 10.1007/s11886-019-1220-y. PMID: 31673869
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
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
Cirrhotic cardiomyopathy: Implications for liver transplantation.
Liu H, Jayakumar S, Traboulsi M, Lee SS
Liver Transpl 2017 Jun;23(6):826-835. doi: 10.1002/lt.24768. PMID: 28407402
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

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
Should We Test for Diastolic Dysfunction? How and How Often?
Litwin SE, Zile MR
JACC Cardiovasc Imaging 2020 Jan;13(1 Pt 2):297-309. Epub 2019 Jun 12 doi: 10.1016/j.jcmg.2019.02.029. PMID: 31202743
Scimitar syndrome.
Ielasi A, Latib A, Agricola E, Montorfano M, Colombo A
J Cardiovasc Med (Hagerstown) 2011 Mar;12(3):176-7. doi: 10.2459/JCM.0b013e328332f3e2. PMID: 20093947
The hemi-Fontan operation.
Jacobs ML, Pourmoghadam KK
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2003;6:90-7. doi: 10.1053/pcsu.2003.50007. PMID: 12740775
Angiomyolipoma of kidney.
Eble JN
Semin Diagn Pathol 1998 Feb;15(1):21-40. PMID: 9503504

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
Should We Test for Diastolic Dysfunction? How and How Often?
Litwin SE, Zile MR
JACC Cardiovasc Imaging 2020 Jan;13(1 Pt 2):297-309. Epub 2019 Jun 12 doi: 10.1016/j.jcmg.2019.02.029. PMID: 31202743
Cirrhotic cardiomyopathy: Implications for liver transplantation.
Liu H, Jayakumar S, Traboulsi M, Lee SS
Liver Transpl 2017 Jun;23(6):826-835. doi: 10.1002/lt.24768. PMID: 28407402
Scimitar syndrome.
Ielasi A, Latib A, Agricola E, Montorfano M, Colombo A
J Cardiovasc Med (Hagerstown) 2011 Mar;12(3):176-7. doi: 10.2459/JCM.0b013e328332f3e2. PMID: 20093947

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
Is supine exercise associated with adverse maternal and fetal outcomes? A systematic review.
Mottola MF, Nagpal TS, Bgeginski R, Davenport MH, Poitras VJ, Gray CE, Davies GA, Adamo KB, Slater LG, Barrowman N, Barakat R, Ruchat SM
Br J Sports Med 2019 Jan;53(2):82-89. Epub 2018 Oct 18 doi: 10.1136/bjsports-2018-099919. PMID: 30337348
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
Risk Factors for Coarctation of the Aorta on Prenatal Ultrasound: A Systematic Review and Meta-Analysis.
Familiari A, Morlando M, Khalil A, Sonesson SE, Scala C, Rizzo G, Del Sordo G, Vassallo C, Elena Flacco M, Manzoli L, Lanzone A, Scambia G, Acharya G, D'Antonio F
Circulation 2017 Feb 21;135(8):772-785. Epub 2016 Dec 29 doi: 10.1161/CIRCULATIONAHA.116.024068. PMID: 28034902
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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