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Superior vena cava syndrome(SVCS)

MedGen UID:
52576
•Concept ID:
C0038833
•
Disease or Syndrome
Synonyms: SVC obstruction; SVC syndrome; SVCS
SNOMED CT: Superior vena cava syndrome (63363004)
 
Monarch Initiative: MONDO:0043287

Definition

Obstruction of the blood flow in the superior vena cava caused by a malignant neoplasm, thrombosis, or aneurysm. It is a medical emergency requiring immediate treatment. Signs and symptoms include swelling and cyanosis of the face, neck, and upper arms, cough, orthopnea, and headache. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSuperior vena cava syndrome

Professional guidelines

PubMed

Angioedema and emergency medicine: From pathophysiology to diagnosis and treatment.
Depetri F, Tedeschi A, Cugno M
Eur J Intern Med 2019 Jan;59:8-13. Epub 2018 Sep 13 doi: 10.1016/j.ejim.2018.09.004. PMID: 30220453
Oncologic Emergencies: Recognition and Initial Management.
Higdon ML, Atkinson CJ, Lawrence KV
Am Fam Physician 2018 Jun 1;97(11):741-748. PMID: 30215936
Superior vena cava syndrome--a proposed classification system and algorithm for management.
Yu JB, Wilson LD, Detterbeck FC
J Thorac Oncol 2008 Aug;3(8):811-4. doi: 10.1097/JTO.0b013e3181804791. PMID: 18670297

Recent clinical studies

Etiology

Endovascular Stenting in Superior Vena Cava Syndrome: A Systematic Review and Meta-analysis.
Aung EY, Khan M, Williams N, Raja U, Hamady M
Cardiovasc Intervent Radiol 2022 Sep;45(9):1236-1254. Epub 2022 Jul 12 doi: 10.1007/s00270-022-03178-z. PMID: 35821122Free PMC Article
Lead-related superior vena cava syndrome: Management and outcomes.
Arora Y, Carrillo RG
Heart Rhythm 2021 Feb;18(2):207-214. Epub 2020 Sep 11 doi: 10.1016/j.hrthm.2020.09.006. PMID: 32920177
Endovascular Therapy for Central Venous Thrombosis.
Gwozdz AM, Silickas J, Smith A, Saha P, Black SA
Methodist Debakey Cardiovasc J 2018 Jul-Sep;14(3):214-218. doi: 10.14797/mdcj-14-3-214. PMID: 30410652Free PMC Article
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
Superior vena cava syndrome: etiology, diagnosis, and treatment.
Baker GL, Barnes HJ
Am J Crit Care 1992 Jul;1(1):54-64. PMID: 1307879

Diagnosis

Endovenous and Operative Treatment of Superior Vena Cava Syndrome.
Kalra M, Sen I, Gloviczki P
Surg Clin North Am 2018 Apr;98(2):321-335. doi: 10.1016/j.suc.2017.11.013. PMID: 29502774
Oncologic Mechanical Emergencies.
Khan UA, Shanholtz CB, McCurdy MT
Hematol Oncol Clin North Am 2017 Dec;31(6):927-940. doi: 10.1016/j.hoc.2017.08.001. PMID: 29078930
Cardiovascular Oncologic Emergencies.
Shenoy S, Shetty S, Lankala S, Anwer F, Yeager A, Adigopula S
Cardiology 2017;138(3):147-158. Epub 2017 Jun 28 doi: 10.1159/000475491. PMID: 28654925
Oncologic mechanical emergencies.
Khan UA, Shanholtz CB, McCurdy MT
Emerg Med Clin North Am 2014 Aug;32(3):495-508. Epub 2014 Jun 7 doi: 10.1016/j.emc.2014.04.001. PMID: 25060246
Clinical practice. Superior vena cava syndrome with malignant causes.
Wilson LD, Detterbeck FC, Yahalom J
N Engl J Med 2007 May 3;356(18):1862-9. doi: 10.1056/NEJMcp067190. PMID: 17476012

Therapy

Malignant Superior Vena Cava Syndrome: A Scoping Review.
Wright K, Digby GC, Gyawali B, Jad R, Menard A, Moraes FY, Wijeratne DT
J Thorac Oncol 2023 Oct;18(10):1268-1276. Epub 2023 May 3 doi: 10.1016/j.jtho.2023.04.019. PMID: 37146753
Interventions for superior vena cava syndrome.
Sen I, Kalra M, Gloviczki P
J Cardiovasc Surg (Torino) 2022 Dec;63(6):674-681. doi: 10.23736/S0021-9509.22.12448-1. PMID: 36469045
Endovascular Stenting in Superior Vena Cava Syndrome: A Systematic Review and Meta-analysis.
Aung EY, Khan M, Williams N, Raja U, Hamady M
Cardiovasc Intervent Radiol 2022 Sep;45(9):1236-1254. Epub 2022 Jul 12 doi: 10.1007/s00270-022-03178-z. PMID: 35821122Free PMC Article
Cardiovascular Oncologic Emergencies.
Shenoy S, Shetty S, Lankala S, Anwer F, Yeager A, Adigopula S
Cardiology 2017;138(3):147-158. Epub 2017 Jun 28 doi: 10.1159/000475491. PMID: 28654925
Oncologic emergencies.
McCurdy MT, Shanholtz CB
Crit Care Med 2012 Jul;40(7):2212-22. doi: 10.1097/CCM.0b013e31824e1865. PMID: 22584756

Prognosis

Radiotherapy for extensive-stage small-cell lung cancer in the immunotherapy era.
Li H, Zhao Y, Ma T, Shao H, Wang T, Jin S, Liu Z
Front Immunol 2023;14:1132482. Epub 2023 Aug 28 doi: 10.3389/fimmu.2023.1132482. PMID: 37701437Free PMC Article
Superior vena cava syndrome-induced hemoptysis.
Al Saadi W, Al Khalili H, Al Hajriy M
Am J Med Sci 2023 Feb;365(2):205-211. Epub 2022 Sep 22 doi: 10.1016/j.amjms.2022.07.021. PMID: 36152811
Lead-related superior vena cava syndrome: Management and outcomes.
Arora Y, Carrillo RG
Heart Rhythm 2021 Feb;18(2):207-214. Epub 2020 Sep 11 doi: 10.1016/j.hrthm.2020.09.006. PMID: 32920177
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
Oncologic emergencies.
McCurdy MT, Shanholtz CB
Crit Care Med 2012 Jul;40(7):2212-22. doi: 10.1097/CCM.0b013e31824e1865. PMID: 22584756

Clinical prediction guides

Clinical analysis of 48 cases of malignant superior vena cava syndrome.
Sun M, Chen X, Li H, Zhang X, Wang X, Zheng R, Li G, Wang L, Li D
World J Surg Oncol 2021 Jun 23;19(1):185. doi: 10.1186/s12957-021-02300-8. PMID: 34162380Free PMC Article
Superior vena cava syndrome during veno-venous extracorporeal membrane oxygenation for COVID-19.
Kopanczyk R, Al-Qudsi OH, Ganapathi AM, Potere BR, Pagel PS
Perfusion 2021 Sep;36(6):630-633. Epub 2021 Jan 9 doi: 10.1177/0267659120987973. PMID: 33427042
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
Cardiac tamponade.
Knoop T, Willenberg K
Semin Oncol Nurs 1999 Aug;15(3):168-73. doi: 10.1016/s0749-2081(99)80004-7. PMID: 10461701
Oncologic emergencies.
Kelly KM, Lange B
Pediatr Clin North Am 1997 Aug;44(4):809-30. doi: 10.1016/s0031-3955(05)70531-9. PMID: 9286286

Recent systematic reviews

Endovascular Stenting in Superior Vena Cava Syndrome: A Systematic Review and Meta-analysis.
Aung EY, Khan M, Williams N, Raja U, Hamady M
Cardiovasc Intervent Radiol 2022 Sep;45(9):1236-1254. Epub 2022 Jul 12 doi: 10.1007/s00270-022-03178-z. PMID: 35821122Free PMC Article
Non-conventional vascular accesses for the management of superior vena cava syndrome in patients with Intestinal Failure. Case series and systematic review.
Pérez Illidge LC, Ramisch D, Valdivieso L, Guzman C, Antoni D, Rumbo C, Trentadue J, Solar H, Gentilini MV, Gondolesi G
Clin Nutr ESPEN 2021 Oct;45:275-283. Epub 2021 Sep 9 doi: 10.1016/j.clnesp.2021.08.008. PMID: 34620329
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
Steroids, radiotherapy, chemotherapy and stents for superior vena caval obstruction in carcinoma of the bronchus: a systematic review.
Rowell NP, Gleeson FV
Clin Oncol (R Coll Radiol) 2002 Oct;14(5):338-51. doi: 10.1053/clon.2002.0095. PMID: 12555872
Steroids, radiotherapy, chemotherapy and stents for superior vena caval obstruction in carcinoma of the bronchus.
Rowell NP, Gleeson FV
Cochrane Database Syst Rev 2001;(4):CD001316. doi: 10.1002/14651858.CD001316. PMID: 11687105

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