Therapy should be causative. Syndrome management recognizes different levels of priority
multidisciplinary approach and symptoms relief is often the first objective of complex care.
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The therapeutic plan is usually targeted to clinical palliation. In fact, most cases are
diagnosed as advanced-stage malignancies.
The patient must immediately assume an orthostatic position. Other supportive treatments
are usually promptly established; oxygen, diuretics, and steroids are also suggested. The
risk of an overlying thrombosis is particularly high and anticoagulant therapy should be
introduced.
In case of malignancy, the treatment can have palliative or, rarely, curative intent.
Chemotherapy is usually employed in lymphomas, small-cell lung cancer and germ cell
tumors. Besides chemotherapy, radiotherapy is widely used in the treatment of non-small cell
lung cancer. Radiation therapy can obtain good results but can also produce an initial
inflammatory response with a possible temporary worsening [28,29]. Some cases must be
approached as an emergency. In this type of situation, the treatment of choice is usually
endovascular with the aim of restoring blood flow as soon as possible. The acute life-
threatening presentation is the only situation in which radiotherapy before histological
diagnosis can be considered. However, this approach should be avoided, whenever possible.
Endovascular stenting provides fast functional relief. It is the best option in an emergency
and sometimes the clinical benefit is immediate. It is also advocated in the case of chemo-
radiotherapy non-responders [3].
Surgery has a central role in the diagnosis but rarely in the therapy. A SVC resection and
reconstruction is not often recommended and is a demanding procedure. The main proposal
for SVC resection is direct infiltration in thymomas or in N0-N1 non-small cell lung cancer.
In the case of infiltration of less than 30% of the SVC circumference, direct suture is favored
(Figure 7). Larger involvements require a prosthetic repair. Different methods of SVC repair
have been investigated using different materials (Figures 8, 9, 10a-b). Armoured PTFE grafts
and biologic material are the preferred choices. Morbidity after SVC surgical procedures is
high and the post-operative care must be intensive [4]. Long-term patency of a SVC by-pass
graft is uncertain but, usually, the slow onset of the graft thrombosis favors the
development of effective collateral circulation.
Fig. 7. SVC resection for limited infiltration by a right upper lobe NSCLC. The moderate
stenosis following the direct SVC suture did not have hemodynamic consequences, in this
patient.
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Fig. 8. Graft reconstruction by end-to-end anastomosis between proximal and distal SVC.
Fig. 9. Graft reconstruction of SVC by end-to-end anastomosis between the right
brachiocephalic vein and the SVC.
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Fig. 10a. Graft reconstruction of SVC by end-to-end anastomosis between the left
brachiocephalic vein and the SVC.
Fig. 10b. Armoured PTFE reconstruction of SVC by end-to-end anastomosis between the left
brachiocephalic vein and the SVC.
Artworks by Walter Santilli R.N. and Elisa Scarnecchia M.D.
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