1. Superior vena cava syndrome (SVCS) prof. L. Grozdinski assoc. prof
Superior vena cava syndrome (SVCS) prof. L. Grozdinski assoc. prof. Ivo Petrov dr Nikolay Iliev CITY Clinic - Sofia

2. Definition
Vena cava superior syndrome is a group of symptoms caused by a disturbance of the blood flow in it, due to a stenosis or thrombosis
First described by William Hunter in 1757 in a patient with syphilistic aneurism of ascending aorta

4. Approximately 15 000 new cases per year only in USA
Epidemiology
Approximately new cases per year only in USA

5. Etiologic Factors: 90% malignant conditions ;
~50% - non–small-cell lung cancer;
~25% - small-cell lung cancer;
~10%- lymphoma;
- 35% - thrombosis – implantation of intravascular devices - catheters and pacemakers.

6. Signs and symptoms Facial edema 60 - 100%
Distended neck veins %
Distended chest veins %
Dyspnea %

7. Clinical diagnosis
In most cases, symptoms are progressive over several weeks;
In some cases – symptoms may improve as collateral circulation develops;
The severity of the symptoms is important in determining the urgency of intervention

8. Management
Most data regarding management are from case series; randomized trials are scarce
Management is guided by:
severity of the symptoms
underlying malignant conditions
anticipated response to treatment

9. Radioterapy
Complete relief of the symptoms of obstruction of the superior vena cava in 78% of patients with small-cell lung cancer and 63% of those with non–small-cell lung cancer.
BUT:
Requires a tissue diagnosis
Objective criterias of the change in vena caval obstruction are not paralleled criterias of symptomatic improvement based on patients’ reports.
Completely relief of vena caval obstruction as measured on serial venograms was noted in 31% of the patients and partial relief in 23% of the patients.
Development of collateral circulation may contribute to improvement of symptoms

10. Systemic Chemotherapy
Complete relief of symptoms in approximately 80% of patients with non-Hodgkin’s lymphoma or small-cell lung cancer and in 40% of those with non–small-cell lung cancer
BUT: review of 2 randomized studies and 44 observational studies concluded that patients with lung cancer have no clinically significant difference in the rate of relief from the superior vena cava syndrome whether chemotherapy, radiotherapy, or chemotherapy with radiotherapy was used

11. Surgery
Sternotomy or thoracotomy with extensive resection and reconstruction of the superior vena cava
Operative mortality - ~ 5%; patency rates - of 80% to 90%
10% reocclusion
BUT: some patients with severe SVCS cannot undergo mediastinoscopy/anesthetize because of their inability to maintain a supine position;

12. Placement of an Intravascular Stent
The stent can be placed before a tissue diagnosis is available
Useful procedure for patients with severe symptoms such as respiratory distress that require urgent intervention
Strongly considered for patients with mesothelioma, which tends not to respond well to chemotherapy or radiation
More prompt relief of symptoms than in radiation or chemotherapy - cyanosis is usually relieved within hours, and edema resolves within 48 to 72 hours in most series (response rate - 75 to 100%)

13. Placement of an Intravascular Stent
Technical success rate – %
Complications of stent placement 3-7% - infection, pulmonary embolus, stent migration, hematoma at the insertion site, bleeding, perforation, recurrent laryngeal palsy
Late complications - bleeding (1% to 14% of patients) and death (1% to 2% of patients) due to anticoagulation

14. Placement of an Intravascular Stent
Rate of relapse - 11%. However 78% of these relapses were successfully managed by repeated intravascular intervention
Patients with brain metastasis may undergo stent placement because of the potential of the superior vena cava syndrome to exacerbate cerebral edema. In these cases at least a temporary anticoagulation is needed which is associated with cerebral hemorrhage
However there are no absolute contraindication

15. Case 1
66 y old patient
May 2010 – small cell lung cancer was diagnosed
April 2011 – venography - high grade stenosis of v. brachiocephalica billateral, PTA and stent implantation of v. brachiocephalica sinstra and baloon angioplasty of v. brachiocephalica dextra
July 2011 – CT scan data for restenosis of v. brachiocephalica dextra
August 2011 – venography - 90% restenosis of v. brachiocephalica dextra – after predilalatation was implanted stent.

19. Case 2
59 y.o. patient
June diagnosed with small-cell lung cancer
August angiographic data of subtotal occlusion of VCS.
PTA – baloon dilatation and stent implantation

22. Case 3 66 y old patient 2000 г.- non-Hodgkin lymphoma was diagnosed.
Later thrombosis of the right subclavian vein
March 2011 г.- СТ data – partial thrombosis of truncus brachiocephalicus
August 2011 г.- venography – subtotal occlusion of v. braciocephalica sin. and occlusion of v. braciocephalica dex.
After balloon predialatation - v. braciocephalica sin. was implanted stent

25. Conclusions
In patients with life-threatening symptoms or signs of obstruction of the superior vena cava, the placement of an intravascular stent can provide rapid relief of the symptoms
Outcomes and complications of PTA compared with chemotherapy/radiotherapy (associated with risks of excessive toxic effect) are in favor of PTA
Stent placement does not reduce the likelihood of cure of the underlying malignant condition and should not compromise the choice of appropriate therapy
There are not routine imaging and anticoagulation protocols. Expert’s consensus on definition of clinical and imaging follow up algorithms is needed