Superior Vena Cava Obstruction

Aditya Rachakonda, PGY4

December 8, 2014
 Objectives
• Overview
• Anatomy
• Etiology
• Clinical Findings
• Diagnostic Evaluation
• Conservative Therapy
• Endovascular Treatment
• Surgical Treatment
 Overview
• Symptoms of venous congestion of head/neck 2/t
occlusion of the SVC or innominate vein develops
in 15,000 pts/year
• SVC syndrome caused by malignant tumors of
lung and mediastinum in 60% of cases
• Incidence of non-malignant cases 2/t intravenous
devices is increasing
• Treatment in pts w/ advanced malignant disease
is palliative, but is curative in those w/ benign
disease
 Anatomy
• SVC is the primary venous drainage system from the
head, neck, upper extremities, and upper thorax
• Approx. 6 to 8 cm long
• Extends from junction of the R and L innominate veins
to the R atrium
• Located in the middle mediastinum
• Surrounded by relatively rigid structures
– Trachea, R bronchus, sternum, aorta,
paratracheal/perihilar lymph nodes
• Low-pressure, thin-walled structure  easily
compressed by adjacent structures
 Etiology
• First case of SVC obstruction was due to aortic aneurysm -
described by William Hunter in 1757
• Incidence of SVCS 2/t tuberculosis, syphilitic mediastinitis,
aortitis has decreased in the 20th century due to anti-
microbial tx
• Currently, MCC of SVCS is lung cancer w/ mediastinal
lymphadenopatny and primary mediastinal malignancy
– Non-small cell lung ca (50%)
– Small-cell lung ca (22%)
– Lymphoma (12%)
– Metastatic ca (9%)
– Thymoma (2%)
– Plasmacytoma
 Etiology
• Benign disease is the cause of SVCS in 40% of
cases
– Mediastinal fibrosis and granulomatous fungal disease
(histoplasmosis) have historically been MCC of benign
obstruction
– Rapid increase in the use of indwelling venous
catheters and cardiac pacemakers has resulted in a
greater number of pts w/ benign etiology
•  5 million central venous catheters and 500K pacemakers
are implanted in the US annualy
• Associated with upper extremity or central deep vein
obstruction in 7 – 33% of cases
– SVCS occurs in 1 – 3% of these cases
 Etiology
• Other important causes for SVC obstruction:
– Previous radiotherapy to the mediastinum
– Retrosternal goiter
– Aortic dissection
• Risk of SVCS is increased in pts w/
thrombophilia:
– Factor V Leiden
– Antithrombin III deficiency
– Protein C or S deficiency
Causes of SCVS
 Clinical Findings
• Signs and symptoms determined by duration and extent of
occlusion and amount of collateral development
• Feeling of fullness in head/neck that is exacerbated by bending over
or lying flat
– Severity of disease can be judged by # of pillows needed to sleep
comfortably
• Dyspnea
• Orthopnea
• Headache/dizziness
• Syncope
• Visual symptoms
• Signs/symptoms of malignant SVCS: hemoptysis, hoarseness,
dysphagia, weight loss, cervical lymphadenopathy
 Clinical Findings
• Physical Exam:
– Prominent chest wall collaterals
– Dilated neck veins
– Swelling of the face, neck, and eyelids
– Ecchymosis and cyanosis of the face
– Mild to moderate upper extremity swelling
Signs/Symptoms
 Diagnostic Evaluation
• Clinical diagnosis suggested by H & P, can be
confirmed by diagnostic imaging
– Plain film radiographs – mediastinal widening, R hilar
mass, pleural effusion, upper lobe collapse
• Normal CXR does not preclude dx of SVCS
– Ultrasonography
• Direct visualization of SVC not possible
• Subclavian and IJ vein can be studied for indirect evidence of
SVC obstruction
– Respiratory variation in flow is lost
– Increased diameter of deep veins
– Visualization of numerous collateral vessels
 Diagnostic Evaluation
• CTA
– Can demonstrated location and extent of obstruction
– Can distinguish benign and malignant causes
– Can identify collateral pathways
• Azygos-hemiazygos
• Internal mammary/superior, inferior epigastric veins
• Lateral thoracic venous system
• Contrast-enhanced venography
– Gold-standard
– Used to provide “roadmap” prior to reconstructive procedures
– Depicts presence and direction of venous collateral flow

*Note: during venography, only veins and collateral pathways between

injection site and RA are visualized – IJ, frequently used for inflow for
surgical bypass, is not visualized
35 y/o M w/ histoplasmosis, 3yr h/o progressive facial and upper-
extremity swelling
CTA shows narrowed SVC w/ adjacent calcified lymph nodes
Sonogram shows markedly dampened venous waveforms with a loss in
respiratory variation
 Stanford and Doty Classification of SVCS
• Based on extent of obstruction and direction of collateral flow
– Type I: high-grade SVC stenosis but normal direction of flow through
SVC and azygos vein; increased collateral circulation thru hemiazygos
and accessory azygos pathway
– Type II: greater than 90% stenosis or occlusion of the SVC but a patent
azygos vein with normal direction of flow
 Stanford and Doty Classification of SVCS
• Type III: complete occlusion of the SVC with retrograde flow
through the azygos and hemiazygos veins
• Type IV: extensive occlusion of the SVC, innominate, and azygos
veins with chest wall and epigastric venous collaterals
 Conservative Therapy
• Used first in every pt to relieve symptoms of venous
congestion and to decrease progression of venous
thrombosis
– Elevation of head w/ pillows while sleeping
– Modify daily activities to avoid bending or supine positions
– Avoid constricting garments or tight collars
– Diuretic agents to decrease excessive edema in head/neck
– Acute SVCS 2/t malignant disease  IV heparin or LMWH
followed by warfarin to prevent recurrence, protect
collateral circulation
– Treat the underlying cause
• Mediastinal malignancy  irradiation, chemotherapy, or
combinaton
 Endovascular Treatment
• First line of treatment for SVCS – avoids median
sternotomy needed for surgical reconstruction
• Indicated in pts who do not respond to medical therapy
• In contrast to chemoradiation tx for malignant SCVS,
endovascular stenting establishes immediate luminal
patency and provides rapid symptomatic relief
– Has been used prior to chemorads in malignant SCVS with
good success
• In pts w/ SVC obstruction 2/t intraluminal thrombosis,
thrombolytic therapy can be effective
 Endovascular Technique
• Access is typically obtained via femoral vein,
but brachial or IJ access is also used
– If complete occlusion of SVC or brachiocephalic
veins, additional b/l upper extremity access via
brachial should be used to facilitate
catheterization of the occlusion
– Dual access enables antegrade and retrograde
venography, facilitates canalization of the lesion
 Endovascular Technique
• Femoral access w/ 9-Fr introducer sheath
• 260cm Bentson guide wire, followed by a pigtail catheter placed
into the SVC
• Venography of the SVC and brachiocephalic veins is performed
• Traverse the lesion using a hydrophilic GW, exchange for a stiff wire
(Amplatz or Lunderquist) for balloon or stent delivery
– If unable to cross SVC occlusion or high-grade lesion, can place a
thrombolytic infusion catheter for bolus or continuous delivery for a
certain period of time
• Systemic heparinization followed by balloon-predilation
• Angioplasty alone yields poor long term results 2/t high rates of
restenosis  stenting is the preferred treatment
– Balloon-expanding stent
– Self-expanding stent
Pt w/ symptomatic SVC
syndrome

A – High grade stenosis on

venogram
B – Luminal patency re-
established w/ the
placement of a Palmaz
stent
C – CXR demonstrates
location of the stent
 Thrombolytic Therapy
• Can lead to complete clot resolution or can partially
recannalize the thrombus to allow crossing the lesion
• Thrombolytic therapy early after onset of symptoms
leads to an improved response
• Non-malignant SVCS responds more favorably to
thrombolytic therapy because of subacute nature of
lesion
• Many physicians perform lytic therapy before stenting
with good results
– In cases of thrombotic occlusion, pharmacomechanical
thrombectomy allows reduction of thrombolytic dose and
increases the efficacy of mechanical thrombectomy
 Stenting
• Angioplasty alone:
– Results in vessel recoil
– May be acceptable for short segment disease, but most pts w/ SVCS have extensive disease
requiring stenting
• Most commonly used stents:
– Z-stent (Cook)
• Rigid, self-expanding; highest radial force in middle segment
– Palmaz stent (Cordis)
• Balloon-expandable stent w/ high radial force; precise deployment
– SMART stent (Cordis)
• Self-expanding, nitinol stent
– Wallstent (Boston Scientific)
• Most widely used
• Self-expanding with longitudinal flexibility, ideal for long, tortuous lesions

• Covered stents used with limited success in the SVC

– Initially proposed to limit tumor ingrowth
– Thought to have less predisposition to development on neointimal hyperplasia
– Not routinely used because can cover important collaterals, resulting in worsening of
symptoms especially when stent graft occludes
 Surgical Treatment
• Indications:
– Pts w/ benign disease who have extensive chronic
venous thrombosis (type III or IV)
– Pts w/ less extensive disease (type I or II) who have
failed endovascular therapy
• Pts w/ malignant tumors should undergo
reconstruction via median sternotomy only if
their life expectancy is greater than 1 year
– Extra-anatomic subcutaneous bypass between jugular
vein and femoral vein with composite saphenous vein
or PTFE is an option if symptoms are severe and
endovascular treatment has failed
 Graft Materials
• Large diameter autologous vein is not
available to use as a conduit
• Greater Saphenous Vein Graft
– Poor size match
– Suitable for extra-anatomic reconstruction; both
saphenous veins are harvested and sutured
together
– To prevent external compression, some authors
have placed the composite GSV graft into an
externally supported ePTFE graft
 Graft Materials
• Femoral or femoropopliteal vein graft
– Excellent suitability in terms of size and length
– If pt has underlying thrombotic abnormalities,
removal of deep leg vein can result in moderate
edema/pain  may progress to chronic venous
insufficiency
• Spiral Saphenous Vein Graft
– First described in animal models; first used in pts by
Doty
– Autologous vein, low thrombogenicity
– Length is limited by available saphenous vein,
diameter can be adjusted as needed
• Saphenous vein is removed,
distended w/ papaverine soln,
opened longitudinally
• Valves are excised
• Wrapped around a 32-36Fr chest
tube
• Edges of the vein are sutures
together
• Length of saphenous vein to be
harvested to create graft is based
on:
– l = RL/r
– Harvesting from groin to knee
usually results in 10cm long
SSVG
 Graft Materials
• ePTFE Grafts
– Most commonly used prosthetic for large vein
reconstruction
– Flow through innominate vein exceeds 1000mL/min 
short, large diameter (10-14mm) grafts have excellent long
term patency
• If peripheral anastomosis is performed w/ the subclavian vein,
venous inflow is significantly less and an AVF in the arm may be
needed to improve patency
• For an IJ – atrial appendage bypass, a 12mm ePTFE graft is suitable
(AVF to graft is not required)
– Externally supported grafts are a good choice in pts w/
malignant disease
• Recurrent tumor is more likely to compress and occlude vein grafts
A – Venography demonstrates type III SVC occlusion w/ retrograde flow in the azygous
vein
B – Spiral saphenous vein graft (constructed using non-penetrating vascular clips) used
for L IJ/R atrial appendage bypass
C – Post-op venogram demonstrating a patent graft. Graft is patent is pt is asymptomatic
at 3 years.
Cumulative Secondary Patency of Vein
and ePTFE Grafts for SVC
reconstruction
 References
1) Cronenwett, Jack L., K. Wayne. Johnston, and Robert B.
Rutherford. "Chapter 61 – Superior Vena Cava Obstruction:
Surgical Treatment." Rutherford's Vascular Surgery. Philadelphia,
PA: Saunders/Elsevier, 2010.
2) Cronenwett, Jack L., K. Wayne. Johnston, and Robert B. Rutherford.
"Chapter 62 – Superior Vena Cava Obstruction: Endovascular
Treatment." Rutherford's Vascular Surgery. Philadelphia, PA:
Saunders/Elsevier, 2010.
3) Nickloes, Todd A. "Superior Vena Cava Syndrome." Superior Vena Cava
Syndrome. Medscape Reference, May-June 2012. Oct 2012.
<http://emedicine.medscape.com/article/460865-overview>.