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Obstruction of the superior vena cava

MedGen UID:
277776
•Concept ID:
C1336532
•
Pathologic Function
Synonyms: Obstruction of superior vena cava; Occlusion of superior vena cava; Superior caval vein obstruction; Superior vena cava obstruction; Superior Vena Cava Occlusion; Superior vena cava syndrome
SNOMED CT: Occlusion of superior vena cava (470759007); Superior caval vein obstruction (766949003); Obstruction of superior vena cava (766949003)
 
HPO: HP:0031041

Definition

Blockage of blood flow through the superior vena cava (SVC). Because the venous drainage from the upper extremities, upper thorax and head is obstructed, SVC obstruction presents with symptoms related to engorgement of these areas. Both the degree of SVC compromise and the extent of collateral veins determine the varied clinical presentation, which can be as mild as slight facial and upper extremity edema or as dire as intracranial swelling, seizures, hemodynamic instability and tracheal obstruction. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVObstruction of the superior vena cava

Conditions with this feature

Autoimmune lymphoproliferative syndrome, type III caused by mutation in PRKCD
MedGen UID:
816258
•Concept ID:
C3809928
•
Disease or Syndrome
Autoimmune lymphoproliferative syndrome type III is an autosomal recessive disorder of immune dysregulation. The phenotype is variable, but most patients have significant lymphadenopathy associated with variable autoimmune manifestations. Some patients may have recurrent infections. Lymphocyte accumulation results from a combination of impaired apoptosis and excessive proliferation (summary by Oliveira, 2013). For a general description and a discussion of genetic heterogeneity of ALPS, see 601859.
See: Condition Record
Autoimmune lymphoproliferative syndrome, type III caused by mutation in PRKCD

Professional guidelines

PubMed

Management of superior vena cava syndrome in critically ill cancer patients.
Morin S, Grateau A, Reuter D, de Kerviler E, de Margerie-Mellon C, de Bazelaire C, Zafrani L, Schlemmer B, Azoulay E, Canet E
Support Care Cancer 2018 Feb;26(2):521-528. Epub 2017 Aug 24 doi: 10.1007/s00520-017-3860-z. PMID: 28836006
Superior mediastinal syndrome: emergency management.
Jain R, Bansal D, Marwaha RK, Singhi S
Indian J Pediatr 2013 Jan;80(1):55-9. Epub 2012 Oct 10 doi: 10.1007/s12098-012-0884-8. PMID: 23054856
Lung cancer. Palliative care.
Kvale PA, Simoff M, Prakash UB; American College of Chest Physicians
Chest 2003 Jan;123(1 Suppl):284S-311S. doi: 10.1378/chest.123.1_suppl.284s. PMID: 12527586

Recent clinical studies

Etiology

Learning from the pulmonary veins.
Porres DV, Morenza OP, Pallisa E, Roque A, Andreu J, Martínez M
Radiographics 2013 Jul-Aug;33(4):999-1022. doi: 10.1148/rg.334125043. PMID: 23842969
Obstruction of the superior vena cava after neonatal extracorporeal membrane oxygenation: association with chylothorax and outcome of transcatheter treatment.
Kazanci SY, McElhinney DB, Thiagarajan R, Bergersen L, Wilson JM, Marshall AC, Lock JE, Mullen MP
Pediatr Crit Care Med 2013 Jan;14(1):37-43. doi: 10.1097/PCC.0b013e31825b5270. PMID: 23295835
Superior vena cava syndrome as tumour presentation.
Pires NF, Morais A, Queiroga H
Rev Port Pneumol 2010 Jan-Feb;16(1):73-88. PMID: 20054509
Surgical reconstruction of the superior vena cava system: indications and results.
Picquet J, Blin V, Dussaussoy C, Jousset Y, Papon X, Enon B
Surgery 2009 Jan;145(1):93-9. Epub 2008 Sep 19 doi: 10.1016/j.surg.2008.08.003. PMID: 19081480
The role of venography and surgery in the management of patients with superior vena cava obstruction.
Stanford W, Doty DB
Ann Thorac Surg 1986 Feb;41(2):158-63. doi: 10.1016/s0003-4975(10)62659-8. PMID: 3947168

Diagnosis

Rapid Fire: Superior Vena Cava Syndrome.
Zimmerman S, Davis M
Emerg Med Clin North Am 2018 Aug;36(3):577-584. Epub 2018 Jun 12 doi: 10.1016/j.emc.2018.04.011. PMID: 30037444
Management of superior vena cava syndrome in critically ill cancer patients.
Morin S, Grateau A, Reuter D, de Kerviler E, de Margerie-Mellon C, de Bazelaire C, Zafrani L, Schlemmer B, Azoulay E, Canet E
Support Care Cancer 2018 Feb;26(2):521-528. Epub 2017 Aug 24 doi: 10.1007/s00520-017-3860-z. PMID: 28836006
Learning from the pulmonary veins.
Porres DV, Morenza OP, Pallisa E, Roque A, Andreu J, Martínez M
Radiographics 2013 Jul-Aug;33(4):999-1022. doi: 10.1148/rg.334125043. PMID: 23842969
Obstruction of the superior vena cava after neonatal extracorporeal membrane oxygenation: association with chylothorax and outcome of transcatheter treatment.
Kazanci SY, McElhinney DB, Thiagarajan R, Bergersen L, Wilson JM, Marshall AC, Lock JE, Mullen MP
Pediatr Crit Care Med 2013 Jan;14(1):37-43. doi: 10.1097/PCC.0b013e31825b5270. PMID: 23295835
Peripheral edema.
Powell AA, Armstrong MA
Am Fam Physician 1997 Apr;55(5):1721-6. PMID: 9105200

Therapy

Impact of bilateral bidirectional Glenn anastomosis on staged Fontan strategy and Fontan circulation.
Imai K, Hoashi T, Okuda N, Ohuchi H, Kurosaki K, Ichikawa H
Eur J Cardiothorac Surg 2021 Oct 22;60(4):930-938. doi: 10.1093/ejcts/ezab162. PMID: 33895850
Tuberculosis lymphadenopathy: A rare etiology of the superior vena cava syndrome.
Mleyhi S, Sandi T, Ben Mrad M, Miri R, Besbes T, Messai M, Ben Omrane S, Kalfat T, Denguir R
J Med Vasc 2021 Feb;46(1):9-12. Epub 2020 Dec 2 doi: 10.1016/j.jdmv.2020.11.001. PMID: 33546823
Obstruction of the superior vena cava after neonatal extracorporeal membrane oxygenation: association with chylothorax and outcome of transcatheter treatment.
Kazanci SY, McElhinney DB, Thiagarajan R, Bergersen L, Wilson JM, Marshall AC, Lock JE, Mullen MP
Pediatr Crit Care Med 2013 Jan;14(1):37-43. doi: 10.1097/PCC.0b013e31825b5270. PMID: 23295835
Lung cancer. Palliative care.
Kvale PA, Simoff M, Prakash UB; American College of Chest Physicians
Chest 2003 Jan;123(1 Suppl):284S-311S. doi: 10.1378/chest.123.1_suppl.284s. PMID: 12527586
Treatment of obstruction of the superior vena cava by combination chemotherapy with and without irradiation in small-cell carcinoma of the bronchus.
Spiro SG, Shah S, Harper PG, Tobias JS, Geddes DM, Souhami RL
Thorax 1983 Jul;38(7):501-5. doi: 10.1136/thx.38.7.501. PMID: 6310812Free PMC Article

Prognosis

Management of superior vena cava syndrome in critically ill cancer patients.
Morin S, Grateau A, Reuter D, de Kerviler E, de Margerie-Mellon C, de Bazelaire C, Zafrani L, Schlemmer B, Azoulay E, Canet E
Support Care Cancer 2018 Feb;26(2):521-528. Epub 2017 Aug 24 doi: 10.1007/s00520-017-3860-z. PMID: 28836006
Superior vena cava syndrome secondary to recurrent coronary artery fistula: A case report and literature review.
Chen PF, Tang L, Liu ZZ, Hu X
Medicine (Baltimore) 2017 Dec;96(51):e9111. doi: 10.1097/MD.0000000000009111. PMID: 29390439Free PMC Article
Obstruction of the superior vena cava after neonatal extracorporeal membrane oxygenation: association with chylothorax and outcome of transcatheter treatment.
Kazanci SY, McElhinney DB, Thiagarajan R, Bergersen L, Wilson JM, Marshall AC, Lock JE, Mullen MP
Pediatr Crit Care Med 2013 Jan;14(1):37-43. doi: 10.1097/PCC.0b013e31825b5270. PMID: 23295835
Surgical reconstruction of the superior vena cava system: indications and results.
Picquet J, Blin V, Dussaussoy C, Jousset Y, Papon X, Enon B
Surgery 2009 Jan;145(1):93-9. Epub 2008 Sep 19 doi: 10.1016/j.surg.2008.08.003. PMID: 19081480
Lung cancer. Palliative care.
Kvale PA, Simoff M, Prakash UB; American College of Chest Physicians
Chest 2003 Jan;123(1 Suppl):284S-311S. doi: 10.1378/chest.123.1_suppl.284s. PMID: 12527586

Clinical prediction guides

Impact of bilateral bidirectional Glenn anastomosis on staged Fontan strategy and Fontan circulation.
Imai K, Hoashi T, Okuda N, Ohuchi H, Kurosaki K, Ichikawa H
Eur J Cardiothorac Surg 2021 Oct 22;60(4):930-938. doi: 10.1093/ejcts/ezab162. PMID: 33895850
CT Features of Vertebral Venous Congestion Simulating Sclerotic Metastases in Nine Patients With Thrombosis of the Superior Vena Cava.
Kara M, Pradel C, Phan C, Miquel A, Arrivé L
AJR Am J Roentgenol 2016 Jul;207(1):80-6. Epub 2016 Apr 11 doi: 10.2214/AJR.15.15756. PMID: 27064458
Surgical and Anatomical Particularities and Difficulties in Histopathological Sampling for Superior Vena Cava Syndrome.
Salahoru P, Lovin CS, Lunguleac T, Mitrofan C, Costache DV, Grigorescu C
Pneumologia 2015 Oct-Dec;64(4):26-7. PMID: 27451591
Superior vena cava syndrome as tumour presentation.
Pires NF, Morais A, Queiroga H
Rev Port Pneumol 2010 Jan-Feb;16(1):73-88. PMID: 20054509
Surgical reconstruction of the superior vena cava system: indications and results.
Picquet J, Blin V, Dussaussoy C, Jousset Y, Papon X, Enon B
Surgery 2009 Jan;145(1):93-9. Epub 2008 Sep 19 doi: 10.1016/j.surg.2008.08.003. PMID: 19081480

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