Superior Vena Cava Syndrome in Emergency Medicine 

  • Author: Michael S Beeson, MD, MBA, FACEP; Chief Editor: David FM Brown, MD   more...
 
Updated: Dec 7, 2010
 

Background

Superior vena cava (SVC) syndrome (SVCS) is characterized by gradual, insidious compression/obstruction of the superior vena cava. Although the syndrome can be life threatening, its presentation is often associated with a gradual increase in symptomatology. For this reason, diagnosis is often delayed until significant compression of the superior vena cava has occurred.

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Pathophysiology

Extrinsic compression of the superior vena cava is possible because it has a thin wall coupled with a low intravascular pressure. Because the superior vena cava is surrounded by rigid structures, it is relatively easy to compress. The low intravascular pressure also allows for the possibility of thrombus formation, such as catheter-induced thrombus.

The subsequent obstruction to flow causes an increased venous pressure, which results in interstitial edema and retrograde collateral flow.

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Epidemiology

Frequency

United States

Superior vena cava syndrome is chiefly associated with malignancy. Currently, more than 90% of patients with superior vena cava syndrome have an associated malignancy as the cause. This contrasts with studies in the early 1950s in which a large proportion of cases were nonmalignant. Infectious causes (eg, syphilis, tuberculosis) have decreased because of improvements in antibiotic therapy. Of the nonmalignant causes of superior vena cava syndrome, thrombosis from central venous instrumentation (catheter, pacemaker, guidewire) is an increasingly common event, especially as these procedures become more common.

International

In developing countries, nonmalignant causes of superior vena cava syndrome continue to constitute a significant percentage. Still, superior vena cava syndrome occurs infrequently in the general population.

Mortality/Morbidity

Bronchogenic carcinoma (CA) accounts for more than 80% of cases of superior vena cava syndrome. Even when treated with radiation, only 10% of these patients are alive 30 months after presentation. However, patients with superior vena cava syndrome due to a malignant cause survive only 30 days without radiation.

Race

Superior vena cava syndrome has no racial predilection. However, because of poorer access to adequate health care, some socioeconomic groups have a disproportionately greater representation.

Age

Because most superior vena cava syndromes are caused by bronchogenic carcinoma, the age distribution is skewed strongly toward elderly persons. Nonmalignant causes, as well as lymphoma, tend to affect younger people more than malignancy-associated superior vena cava syndrome. The age range reported in one study was 18-76 years, with a mean age of 54 years.[1]

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Contributor Information and Disclosures
Author

Michael S Beeson, MD, MBA, FACEP  Professor of Emergency Medicine, Northeastern Ohio Universities College of Medicine and Pharmacy; Attending Faculty, Akron General Medical Center

Michael S Beeson, MD, MBA, FACEP is a member of the following medical societies: American College of Emergency Physicians, Council of Emergency Medicine Residency Directors, National Association of EMS Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard S Krause, MD  Senior Clinical Faculty/Clinical Assistant Professor, Department of Emergency Medicine, University of Buffalo State University of New York School of Medicine and Biomedical Sciences

Richard S Krause, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Emergency Medicine, American College of Emergency Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Eddy S Lang, MDCM, CCFP(EM), CSPQ  Associate Professor, Senior Researcher, Division of Emergency Medicine, Department of Family Medicine, University of Calgary Faculty of Medicine; Assistant Professor, Department of Family Medicine, McGill University Faculty of Medicine, Canada

Eddy S Lang, MDCM, CCFP(EM), CSPQ is a member of the following medical societies: American College of Emergency Physicians, Canadian Association of Emergency Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

John D Halamka, MD, MS  Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center

John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Chief Editor

David FM Brown, MD  Associate Professor, Division of Emergency Medicine, Harvard Medical School; Vice Chair, Department of Emergency Medicine, Massachusetts General Hospital

David FM Brown, MD is a member of the following medical societies: American College of Emergency Physicians and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

References
  1. Chen JC, Bongard F, Klein SR. A contemporary perspective on superior vena cava syndrome. Am J Surg. Aug 1990;160(2):207-11. [Medline].

  2. Lanciego C, Pangua C, Chacon JI, et al. Endovascular stenting as the first step in the overall management of malignant superior vena cava syndrome. AJR Am J Roentgenol. Aug 2009;193(2):549-58. [Medline].

  3. Dhaliwal RS, Das D, Luthra S, et al. Management of superior vena cava syndrome by internal jugular to femoral vein bypass. Ann Thorac Surg. Jul 2006;82(1):310-2. [Medline].

  4. [Guideline] Kvale PA, Selecky PA, Prakash UB. Palliative care in lung cancer: ACCP evidence-based clinical practice guidelines (2nd edition). Chest. Sep 2007;132(3 Suppl):368S-403S. [Medline].

  5. Abner A. Approach to the patient who presents with superior vena cava obstruction. Chest. Apr 1993;103(4 Suppl):394S-397S. [Medline].

  6. Armstrong BA, Perez CA, Simpson JR, Hederman MA. Role of irradiation in the management of superior vena cava syndrome. Int J Radiat Oncol Biol Phys. Apr 1987;13(4):531-9. [Medline].

  7. Baker GL, Barnes HJ. Superior vena cava syndrome: etiology, diagnosis, and treatment. Am J Crit Care. Jul 1992;1(1):54-64. [Medline].

  8. Bauset R. Pacemaker-induced superior vena cava syndrome: a case report and review of management strategy. Can J Cardiol. Nov 2002;18(11):1229-32. [Medline].

  9. Courtheoux P, Alkofer B, Al Refai M, et al. Stent placement in superior vena cava syndrome. Ann Thorac Surg. Jan 2003;75(1):158-61. [Medline].

  10. Lanciego C, Chacon JL, Julian A, et al. Stenting as first option for endovascular treatment of malignant superior vena cava syndrome. AJR Am J Roentgenol. Sep 2001;177(3):585-93. [Medline].

  11. Leggio L, Abenavoli L, Vonghia L, et al. Superior vena cava thrombosis treated by angioplasty and stenting in a cirrhotic patient with peritoneovenous shunt. Ann Thorac Cardiovasc Surg. Feb 2008;14(1):60-2. [Medline].

  12. Link MS, Pietrzak MP. Aortic dissection presenting as superior vena cava syndrome. Am J Emerg Med. May 1994;12(3):326-8. [Medline].

  13. Madan AK, Allmon JC, Harding M, et al. Dialysis access-induced superior vena cava syndrome. Am Surg. Oct 2002;68(10):904-6. [Medline].

  14. Queen JR, Berlin J. Superior vena cava syndrome. J Emerg Med. Aug 2001;21(2):189-91. [Medline].

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Patient with a 4-week history of increasing facial edema and known lung cancer.
Chest radiograph of a patient with known superior vena cava syndrome (SVCS) and bronchogenic carcinoma (CA).
Chest radiograph of a 50-year-old woman with complaint of shortness of breath and facial swelling. No previous history of cancer but 30 pack-year history.
CT scan of the same 50-year-old woman in Image 3 with an initial presentation of shortness of breath and facial swelling. This shows a large tumor mass in the right mediastinum nearly surrounding the right main stem bronchus and partially occluding the superior vena cava.
 
 
 
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