Excerpt from Superior Vena Cava Syndrome

Synonyms, Key Words, and Related Terms: SVCS, superior vena cava, SVC, bronchogenic carcinoma, endovascular stenting, endoprostheses, malignancy-associated SVCS, non-malignancy–associated SVCS, superior vena cava syndrome, obstruction of superior vena cava, obstruction of SVC, compression of superior vena cava, compression of SVC, low intravascular pressure, interstitial edema, retrograde collateral flow, thrombus formation

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Background

Superior vena cava syndrome (SVCS) is characterized by gradual, insidious compression/obstruction of the superior vena cava (SVC). Although the syndrome can be life threatening, its presentation is often associated with a gradual increase in symptomatology.

Pathophysiology

Extrinsic compression of the SVC is possible because it has a thin wall coupled with a low intravascular pressure. Because the SVC 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

SVCS is associated chiefly with malignancy. Currently, more than 90% of patients with SVCS 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 SVCS, 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 SVCS continue to constitute a significant percentage. Still, SVCS occurs infrequently in the general population.

Mortality/Morbidity

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

Race

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

Age

Because the majority of SVC 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 SVCS.
The age range reported in one study was 18-76 years, with a mean age of 54 years (Chen, 1990).

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