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.

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.

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]

Clinical Presentation

Chen JC, Bongard F, Klein SR. A contemporary perspective on superior vena cava syndrome. Am J Surg. 1990 Aug. 160(2):207-11. [QxMD MEDLINE Link].
Thakker M, Keteepe-Arachi T, Abbas A, et al. A primary cardiac sarcoma presenting with superior vena cava obstruction. Am J Emerg Med. 2012 Jan. 30(1):264.e3-5. [QxMD MEDLINE Link].
[Guideline] Kvale PA, Selecky PA, Prakash UB. Palliative care in lung cancer: ACCP evidence-based clinical practice guidelines (2nd edition). Chest. 2007 Sep. 132(3 Suppl):368S-403S. [QxMD MEDLINE Link].
Andersen PE, Duvnjak S. Palliative treatment of superior vena cava syndrome with nitinol stents. Int J Angiol. 2014 Dec. 23(4):255-62. [QxMD MEDLINE Link]. [Full Text].
Cui J, Kawai T, Irani Z. Catheter-directed thrombolysis in acute superior vena cava syndrome caused by central venous catheters. Semin Dial. 2015 Sep-Oct. 28(5):548-51. [QxMD MEDLINE Link].
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. 2009 Aug. 193(2):549-58. [QxMD MEDLINE Link].
Sato Y, Inaba Y, Yamaura H, Takaki H, Arai Y. Malignant inferior vena cava syndrome and congestive hepatic failure treated by venous stent placement. J Vasc Interv Radiol. 2012 Oct. 23(10):1377-80. [QxMD MEDLINE Link].
Mokry T, Bellemann N, Sommer CM, et al. Retrospective study in 23 patients of the self-expanding sinus-XL stent for treatment of malignant superior vena cava obstruction caused by non-small cell lung cancer. J Vasc Interv Radiol. 2015 Mar. 26(3):357-65. [QxMD MEDLINE Link].
Dhaliwal RS, Das D, Luthra S, et al. Management of superior vena cava syndrome by internal jugular to femoral vein bypass. Ann Thorac Surg. 2006 Jul. 82(1):310-2. [QxMD MEDLINE Link].
Abner A. Approach to the patient who presents with superior vena cava obstruction. Chest. 1993 Apr. 103(4 Suppl):394S-397S. [QxMD MEDLINE Link].
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. 1987 Apr. 13(4):531-9. [QxMD MEDLINE Link].
Baker GL, Barnes HJ. Superior vena cava syndrome: etiology, diagnosis, and treatment. Am J Crit Care. 1992 Jul. 1(1):54-64. [QxMD MEDLINE Link].
Bauset R. Pacemaker-induced superior vena cava syndrome: a case report and review of management strategy. Can J Cardiol. 2002 Nov. 18(11):1229-32. [QxMD MEDLINE Link].
Courtheoux P, Alkofer B, Al Refai M, et al. Stent placement in superior vena cava syndrome. Ann Thorac Surg. 2003 Jan. 75(1):158-61. [QxMD MEDLINE Link].
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. 2001 Sep. 177(3):585-93. [QxMD MEDLINE Link].
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. 2008 Feb. 14(1):60-2. [QxMD MEDLINE Link].
Link MS, Pietrzak MP. Aortic dissection presenting as superior vena cava syndrome. Am J Emerg Med. 1994 May. 12(3):326-8. [QxMD MEDLINE Link].
Madan AK, Allmon JC, Harding M, et al. Dialysis access-induced superior vena cava syndrome. Am Surg. 2002 Oct. 68(10):904-6. [QxMD MEDLINE Link].
Queen JR, Berlin J. Superior vena cava syndrome. J Emerg Med. 2001 Aug. 21(2):189-91. [QxMD MEDLINE Link].
Wan JF, Bezjak A. Superior vena cava syndrome. Emerg Med Clin North Am. 2009 May. 27(2):243-55. [QxMD MEDLINE Link].

Media Gallery

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.