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STATE-OF-THE-ART REVIEW
Superior Vena Cava Syndrome
Abdul Hussain Azizi, MD,
a
Irfan Shafi, MD,
b
Neal Shah, MD,
a
Kenneth Rosenfield, MD,
c
Robert Schainfeld, DO,
c
Akhilesh Sista, MD,
d
Riyaz Bashir, MD
e
ABSTRACT
Superior vena cava (SVC) syndrome comprises a constellation of clinical signs and symptoms caused by obstruction of
blood flow through the SVC. The management of patients with life-threatening SVC syndrome is evolving from radiation
therapy to endovascular therapy as the first-line treatment. There is a paucity of data and societal guidelines with regard
to the management of SVC syndrome. This paper aims to update the practicing interventionalists with the contemporary
and the evolving therapeutic approach to SVC syndrome. In addition, the review will focus on endovascular techniques,
including catheter-directed thrombolysis, angioplasty, and stenting, and their associated complications.
(J Am Coll Cardiol Intv 2020;13:2896–910) © 2020 by the American College of Cardiology Foundation.
Superior vena cava (SVC) syndrome is caused
by the severe obstruction or occlusion of the
SVC and can result in significant morbidity
and mortality (1–3). Malignancy is the most common
cause of SVC obstruction, accounting for approxi-
mately 70% of cases. However, recently the inci-
dence of device related SVC syndrome from
central venous catheters and pacemaker or defibril-
lator leads has been increasing (Table 1)(2,3). The
management of SVC syndrome is evolving. In the
past, radiation therapy (RT) was considered first-
line treatment, particularly in patients with airway
obstruction. However, in recent years, endovascular
therapy (ET) is more frequently used first, or in
combination with RT, to provide rapid relief of clin-
ical symptoms with reduced complications (2). This
paper aims to update the practicing interventional-
ists with the contemporary and the evolving thera-
peutic approach to SVC syndrome. In addition, the
review will focus on endovascular techniques,
including catheter-directed thrombolysis (CDT),
angioplasty, and stenting, and their associated
complications.
ANATOMY OF THE SVC
The SVC is formed from the union of the right and left
brachiocephalic veins, which provide venous
drainage of the head, neck, and upper limbs. The
major tributary of the SVC is the azygos vein, which
courses along the right anterior borders of the
thoracic vertebrae to the level of the tracheal carina
and drains posteriorly into the SVC. Other small
mediastinal veins may also drain directly into the SVC
and become more prominent on imaging in the
presence of SVC obstruction (4).
Familiarity with the anatomical variants of the SVC
is essential for interventionalists, as these can have
implications for performance of endovascular revas-
cularization in some patients. Persistent left SVC is
the most common congenital anomaly, with preva-
lence of about 0.3% to 0.5% in general population
ISSN 1936-8798/$36.00 https://doi.org/10.1016/j.jcin.2020.08.038
From the
a
Department of Internal Medicine, Temple University Hospital, Philadelphia, Pennsylvania, USA;
b
Department of In-
ternal Medicine, Detroit Medical Center, Wayne State University, Detroit, Michigan, USA;
c
Department of Cardiovascular Diseases,
Massachusetts General Hospital, Boston, Massachusetts, USA;
d
Department of Interventional Radiology, NYU Langone Health,
New York, New York, USA; and the
e
Department of Cardiovascular Diseases, Temple University Hospital, Philadelphia, Penn-
sylvania, USA.
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’
institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information,
visit the Author Center.
Manuscript received June 16, 2020; revised manuscript received July 30, 2020, accepted August 18, 2020.
JACC: CARDIOVASCULAR INTERVENTIONS VOL.13,NO.24,2020
ª2020 BY THE AMERICAN COLLEGE OF CARDIOLOGY FOUNDATION
PUBLISHED BY ELSEVIER
and up to 5.2% in patients with congenital heart
disease (5). Persistent left SVC coexists with a right
SVC in up to 80% to 90% of cases (6). When a double
SVC is present, the right SVC drains into the right
atrium as usual, while the left SVC drains into the
coronary sinus. Isolated left SVC, which consists of
persistent left SVC with absent right SVC, drains into
the right atrium via an enlarged coronary sinus (7).
ETIOLOGY OF SVC SYNDROME
Historically, prior to the development of antibiotics,
SVC syndrome was predominantly attributed to
syphilitic aortic aneurysms and mediastinal aden-
opathy from tuberculosis (8). Currently, malignancy
accounts for approximately 70% of cases, with
benign causes including device-related SVC syn-
drome accounting for 30% (Table 1)(3). A small
number of benign non–device-related SVC syn-
dromes exist, mainly caused by mediastinal fibrosis
(9). Patients with benign SVC syndrome are usually
younger and, as expected, have a longer life ex-
pectancy (10).
The incidence of device related SVC syndrome has
been increasing due to modern-day use of catheters,
pacemakers, and defibrillators (11). In 1 study, 28% of
all SVC syndromes were related to devices (3). Clin-
ically silent venous thrombosis can occur in up to
30% of patients with pacer leads; however, SVC
obstruction is rare and seen in only 0.1% to 3.3% of
patients (11). Stenosis at the SVC-atrial junction oc-
curs due to fibrin deposition on the surface of the
pacing leads and incorporation into the intima fol-
lowed by vessel wall inflammation, fibrosis,
thrombus formation, and stenosis (11,12). Chronic
mechanical irritation and foreign body reaction
are the primary driving mechanisms. Lead extraction
and subsequent reimplantation also causes
mechanical trauma as additional risk factors
for venous occlusion (12).
CLINICAL PRESENTATION AND
GRADING SYSTEM
Clinical presentation varies depending on the
severity, location, and rapidity of onset of
obstruction and establishment of collateral
veins (Table 2). The most common present-
ing symptoms include facial and neck edema, dis-
tended neck and chest veins, watering eyes, and
dizziness particularly when leaning forward. Pa-
tients may also present with symptoms that are
neurological (headache, blurry vision, decreased
level of consciousness), laryngopharyngeal (tongue
swelling, dyspnea), upper extremities (edema), and
facial (conjunctival/periorbital edema) (8). Patients
also typically describe worsening of their symptoms
in the supine position. Rarely, proximal esophageal
varices may be seen (13).
Some patients with malignant SVC syndrome may
present with life-threatening symptoms of cerebral,
laryngeal, and pharyngeal edema due to sudden
elevation in venous pressures from rapidly occluding
SVC (1,8). SVC obstruction rarely causes hemody-
namic compromise unless there is compression of the
cardiac chamber from an underlying malignancy (2).
Acute mortality owing to SVC syndrome is uncommon
(0.3%) (14); however, the median life expectancy of
patients with SVC syndrome secondary to malignancy
is only 6 months (2).
The severity of SVC syndrome based on the clinical
presentation has been described by Yu et al. (15). The
scoring system proposed by these authors, with
ranges from grade 0 to grade 5, can be helpful in
the diagnostic approach and determination of treat-
ment (Table 3)(15). The Kishi score is another rating
system that was developed to assist in decision
making regarding stent therapy. It includes neuro-
logical, laryngeal, facial, and cardiovascular signs and
symptoms. According to this scale, a Kishi score
of 4 or more warrants endovascular intervention
(Supplemental Table 1)(16).
PATTERNS OF OBSTRUCTION AND
COLLATERAL PATHWAYS
In SVC obstruction, the flow of blood is diverted to
the right atrium through a collateral venous network,
whichcantakeseveralweekstoaccommodatethe
usual blood flow of the SVC. The severity of presen-
tation of SVC syndrome is inversely related to the
development of these collateral veins and the rapidity
HIGHLIGHTS
SVC syndrome is caused by obstruction of
blood flow through the SVC and usually
secondary to malignancy; however,
recently, device-related SVC syndrome is
increasing.
ET is emerging as the first-line therapy
for the treatment of SVC syndrome.
Advances in dedicated venous and or
covered stent technology may lead to
further improvement in durability of
endovascular treatment.
ABBREVIATIONS
AND ACRONYMS
CT =computed tomography
CDT =catheter-directed
thrombolysis
ET =endovascular therapy
IVC =inferior vena cava
RT =radiation therapy
SVC =superior vena cava
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DECEMBER 28, 2020:2896–910 SVC Syndrome
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with which SVC obstruction develops (1). SVC
obstruction typically causes the venous pressure to
rise as high as 20 to 40 mm Hg proximal to the
obstruction. This increased venous pressure produces
symptoms of facial, neck, and chest wall edema (1).
There are 4 main collateral pathways: 1) the azygous
venous system, which is the largest and consists of
azygos, hemiazygos, intercostal, and lumbar veins; 2)
the internal mammary pathway; 3) the lateral thoracic
pathway; and 4) the vertebral venous pathway
(Figure 1)(17).
Anatomic classification of SVC obstruction includes
3 levels of obstruction: obstruction of upper SVC
proximal to the azygos vein, obstruction at the level
of the azygos vein, and obstruction distal to the
azygos vein (Figure 2A). An obstruction cephalad to
the azygos vein causes the blood to return to right
atrium through the ayzgos system and intercostal
veins into the SVC (Figure 2B). In SVC obstruction at
the level of azygos, blood cannot re-enter the SVC
throughtheazygossystemandisforcedtoutilize
other collateral veins, leading into the inferior vena
cava (IVC) and from there into the right atrium
(Figure 2C). In SVC obstruction below the level of
azygos vein, blood will be redirected via a robust
azygos and hemi-azygos system in a retrograde
manner ultimately to the IVC, hence causing less se-
vere symptoms (Figure 2D)(18). However, in many
cases, the brachiocephalic veins (or portions thereof)
are also involved, such that the collateral veins
formed are multiple and may include any of the pre-
viously listed pathways (Figure 2A)(19).
Thereisnosinglestandardforclassifyingthe
various types of SVC obstructions. That stated, the
most widely used is the Stanford method, which
classifies SVC obstruction by venography: type I is
partial obstruction of the supra-azygous SVC with
patent infra-azygos SVC and antegrade flow from the
azygos vein. Type II is near-complete obstruction
(>90%) of supra-azygos SVC with patent azygos vein
with antegrade flow. Type III is complete obstruction
of the SVC with patent and antegrade flow in azygos
vein. Last, type IV is complete obstruction of SVC and
1 or more of the major caval tributaries including the
azygos system (20). The Stanford method was devel-
oped to help identify patients at risk for airway or
cerebral compromise warranting surgical interven-
tion, thus not making it widely applicable in patients
with less severe symptoms.
None of the existing classification methods con-
siders both anatomic location and severity of
obstruction. To address this gap, we propose a clas-
sification system that is based on both location and
severity. When used in conjunction with clinical
symptoms, our system may be useful to guide overall
management and facilitate communication among
clinicians (Figure 3). The proposed classification can
be widely used by other researchers, clinicians and
interventionalists with the hope of validating the
association with various procedural and long-term
outcomes.
DIAGNOSTIC APPROACH
The diagnosis of SVC syndrome is based on the clin-
ical presentation and advanced imaging. Imaging
modalities include chest radiography, contrast-
enhanced computed tomography (CT) scanning,
duplex ultrasound, conventional catheter-based dig-
ital subtraction venography, and magnetic resonance
venography. Contrast-enhanced CT scanning pro-
vides optimal visualization of the SVC and can
localize the extent of venous blockage, differentiate
thrombosis from extrinsic compression, and identify
collateral pathways (21). CT findings include lack of
opacification, presence of an intraluminal filling
defect or narrowing of the SVC, and visualization of
the collateral pathways. The presence of collateral
vessels on contrast-enhanced CT is a very accurate
predictor of clinically relevant and symptomatic SVC
syndrome, while delineation of SVC obstruction alone
is a less specific predictor. Of note, the collateral
TABLE 1 Etiologies of SVC Syndrome
Malignant Causes of SVC Syndrome (70%) Benign Causes of SVC Syndrome (w30%)
Non–smallcelllungcancer(w50%) Central venous catheters, pacemakers, defibrillator, indwelling
hemodialysis catheters (25–30%)
Smallcelllungcancer(w25%) Other benign causes include radiation fibrosis, infection (syp hilis
and tuberculosis—most common causes 50 years ago), idiopathic
mediastinal fibrosis, retrosternal thyroid, aortic aneurysm, benign
tumors, mediastinal hematoma, sarcoidosis, and iatrogenic causes
(w1–5%)
Lymphomas (w10%)
Other cancers including thymoma, primary germ cell neoplasms,
mesothelioma, and solid tumors with mediastinal lymph node
metastasis (e.g., breast cancer) (w15%)
Sources: Friedman et al. (1), Rice et al. (3).
SVC ¼superior vena cava.
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venous system persists on imaging after endovascular
intervention, even when clinical symptoms improve
(22).
Duplex ultrasound of the upper extremities is
useful for evaluating for thrombus in the jugular,
subclavian, and the axillary veins. It is also helpful in
identifying indwelling device-associated thrombi and
for identifying the upper extremity access site for
venography and endovascular intervention. Howev-
er, direct visualization of the SVC and brachiocephalic
veins is challenging, owing to overlying ribs and lung
shadows (21). Digital subtraction venography is the
gold standard for evaluation of SVC obstruction,
including the presence of thrombus. Venography
identifies collateral venous pathways and defines the
severity of obstruction, and enables the inter-
ventionalist to develop a strategy for definitive
revascularization. Intravenous access enables
assessment of the hemodynamic significance of the
blockage, as well as the presence of any congenital
anomalies. The limitation of invasive venography is
the inability, even when combined with intravas-
cular ultrasound, to evaluate the specificcauseof
extrinsic SVC compression (23). Magnetic resonance
venography is an alternative approach in patients
who demonstrate contrast dye allergy, or in cases
where venous access cannot be obtained. Magnetic
resonance venography is equally sensitive and spe-
cific, when compared with conventional venography,
in identifying SVC obstruction (21,24).
TREATMENT APPROACH
The treatment approach in patients with SVC syn-
drome should be multidisciplinary and may include
oncology, pulmonology, radiology, surgery, and
vascular and endovascular specialists. Treatment
options can include chemotherapy with or without
RT, surgical bypass, or ET such as angioplasty,
stenting, and catheter-based thrombus removal
(Central Illustration). For advantages and disadvan-
tages of different treatment modalities, see
Supplemental Table 2.
Initial management for all patients with SVC syn-
drome includes elevation of the head of the bed to
decrease the hydrostatic pressure in the head and
neck. The management of SVC syndrome related to
malignancy is centered on immediate relief of symp-
toms,aswellasspecific treatment of the underlying
cancer. In life-threatening situations, initial stabili-
zation with ABCs (airway, breathing, circulation) is
quickly followed by endovascular recanalization with
or without stenting to quickly address the obstruction
and provide relief of symptoms (25). Parenteral glu-
cocorticoids and loop diuretic agents are commonly
used medications in SVC syndrome, but data are
lacking regarding their efficacy (8). Steroids are often
used as prophylaxis against radiation-induced edema
and are also used in patients with airway compromise
(26). If steroids are used, they should be of high po-
tency and duration should be limited (27).
Management is guided by the severity of the
symptoms and identification of the underlying ma-
lignancy. A histologic diagnosis is critical to provide a
comprehensive tumor- and stage-specifictreatment
plan. Depending on the underlying etiology, some
patients can achieve long-term relapse-free survival
and cure.
RADIATION THERAPY. Traditionally, SVC syndrome
had been viewed as a relative emergency and RT was
considered as the first-line treatment. Urgent initia-
tion of RT was believed to be the fastest way to
relieve the obstruction in patients with life-
threatening symptoms. Recently, RT has been less
frequently used because of the following reasons:
histologic diagnosis after RT is impossible in
approximately 40% of cases. RT leads to symptomatic
relief in about 80% of patients; however, it can take
TABLE 2 Signs and Symptoms of SVC Syndrome
Signs and Symptoms Incidence (%)
Facial edema 60–100
Nonpulsatile distended neck veins 27–86
Distended chest veins 38–67
Dyspnea and cough 23–70
Arm edema 14–75
Hoarseness and/or stridor 0–20
Syncope and/or headache 6–13
Confusion, obtundation 0–5
Signs and symptoms are presented in order of most common to least common.
Adapted from Wilson et al. (2).
SVC ¼superior vena cava.
TABLE 3 Grading of SVC Syndrome per Yu et al. (15)
Grade Finding(s)
0 Asymptomatic: SVC on imaging without symptoms
1 Mild: edema of head or neck
2 Moderate: edema in head or neck with functional impairment
3 Severe: mild or moderate cerebral edema/laryngeal edema, or
diminished cardiac reserve
4 Life-threatening: significant cerebral edema, laryngeal edema,
hemodynamic compromise
5 Fatal: death
SVC ¼superior vena cava.
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as long as 3 days to 4 weeks for the improvement.
Furthermore, up to 20% of patients do not achieve
symptomatic relief. RT reduces tumor burden, but the
benefits are often temporary with 5% to 30% of pa-
tients experiencing recurrence of SVC syndrome
(27,28). In contrast, endovascular stenting results in
more rapid relief of symptoms (29,30).
SURGICAL INTERVENTION. Open surgical interven-
tion, such as bypass grafting and SVC reconstruction,
is reserved for cases of extensive venous thrombosis
or occlusion that are highly symptomatic and not
amenable to endovascular intervention. Open surgi-
cal bypass was once considered a mainstay of treat-
ment for SVC syndrome in patients with a benign
etiology, particularly in those with long life expec-
tancy. The surgical bypass is usually performed from
the innominate or the jugular vein to the right atrial
appendage or the SVC using a spiral saphenous vein
graft (10). Nearly one-half of surgical bypasses
FIGURE 1 Four Main Collateral Pathways UtilizedinSVCObstruction
The azygos system (pink) consists of the azygos, hemiazygos, intercostal, and lumbar veins. Of note, superior vena cava (SVC) obstruction
from infra-azygous occlusion utilizes this pathway, leading to milder symptoms. The internal mammary pathway (blue) consists of internal
thoracic, superior and inferior epigastric, and superficial thoracic veins (not labeled). The lateral thoracic pathway (not labeled) consists of
small veins mainly including the lateral thoracic, thoracoepigastric, superficial circumflex, and femoral veins. The vertebral pathway (purple)
collateralizes to the azygos and internal mammary pathways through the vertebral, intercostal, and lumbar veins. IVC ¼inferior vena cava.
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SVC Syndrome DECEMBER 28, 2020:2896–910
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FIGURE 2 Anatomic Classification of SVC Obstruction
(A) Obstruction of SVC involving the left and right brachiocephalic veins: collateral veins formed can be multiple and may include any of the
described pathways. (B) Obstruction of the SVC proximal to azygos entry point (supra-azygos): blood flow is directed to the azygos through
the right superior intercostal vein. (C) Obstruction of SVC at level of azygos: the azygos venous system cannot be used as a collateral pathway;
other chest wall collateral veins such as superior epigastric and internal mammary veins are formed, and symptoms are usually more severe.
(D) Obstruction of SVC distal to azygos entry point (infra-azygos): retrograde blood flow utilizes the azygos and hemiazygos veins to the IVC
leading to milder symptoms. Abbreviations as in Figure 1.
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ultimately require endovascular intervention to
maintain secondary patency (10).
ENDOVASCULAR THERAPY. Over the last 2 decades,
endovascular intervention with stenting has become
the standard of care for SVC obstruction, for both
benign and malignant etiologies. The benefits of ET
include rapid resolution of symptoms with high
technical success rate and low procedural complica-
tions (Supplemental Figure 1). Additionally, ET does
not adversely affect the outcomes of open surgical
bypass, should patients subsequently need it (10). ET
does not affect the subsequent histologic diagnosis
and can be combined with other treatment modal-
ities including chemotherapy and radiation, if
needed (25).
In the contemporary era, ET is used as the first-line
therapyinmajorityofpatientswithSVCsyndrome,
particularly those presenting with life-threatening
symptoms such as cerebral or laryngeal edema or
postural syncope. Although there are no prospective
randomized studies of ET in treatment of SVC syn-
drome, observational data suggest a robust technical
success rate of 80% to 98%, with symptomatic relief
in >90% of patients. The results also appear to be
durable with a restenosis rate of 4.3% to 29.5%
(average 11.9%) and recurrence rate of 1.2% to 20.5%
(average 10.5%) (Table 4)(25,31–42).
Optimal treatment of pacemaker- or defibrillator-
related SVC syndrome is not well defined, as there is
very little evidence to help guide clinicians. The
FIGURE 3 Proposed Classification of SVC Obstruction Based on Location and Severity
Proposed classification system that considers anatomic location and severity of SVC obstruction. When used in conjunction with clinical
symptoms, it may be used to guide management and facilitate communication among clinicians. SVC ¼superior vena cava.
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presence of leads limits the possible therapeutic ap-
proaches, especially stent deployment. Balloon an-
gioplasty alone has been used to address venous
stenosis from a pacemaker lead; in 1 study, patency
after intervention was noted to be 86% after
12 months (43). In cases in which stenting may be
required to maintain patency, the leads should be
removal prior to stent deployment in order to avoid
entrapment. The incidence of SVC syndrome after
pacemaker or implantable cardioverter-defibrillator
lead placement is a very rare adverse event, and
routine anticoagulation as a preventive measure is
not recommended even in high-risk patients, as the
risks of bleeding outweighs the benefits.
SVC SYNDR OME WI TH THRO MBOS IS. In some cases
of SVC obstruction, there is superimposed throm-
bosis, likely related to stagnation of flow, a hyperco-
agulable state from underlying malignancy, or the
presence of indwelling catheters (Figure 4). In such
patients, the presentation is often acute and during
intervention, the guidewire easily traverses the
occluded segment. Thrombus removal with CDT or
aspiration thrombectomy is recommended prior to
revascularization, in order to prevent pulmonary
embolism and reduce the length of lesion to be
treated (44). Thrombolysis or thrombectomy should
be initiated within 2 to 5 days of symptom onset for
treatment to be effective. After several days, the
ability to achieve successful clot lysis drops signifi-
cantly as the thrombus becomes organized (45). CDT
hasbeenshowntobesafeandeffectiveinpatients
both with and without cancer (40). If patients are at
elevated risk of bleeding and have an absolute
contraindication to thrombolytic agents, then me-
chanical thrombectomy may be preferred (46).
ENDOVASCULAR TECHNIQUES
Most of these procedures are performed in a supine
position in the endovascular suite with local anes-
thesia and conscious sedation. Some patients may
need to have the head elevated or need general
anesthesia with mechanical ventilation depending on
if symptoms worsen by lying flat or the presence of
airway edema, respectively (44,47). During angio-
plasty/stenting, additional opiate medications may be
needed, as dilation of the stricture may cause
discomfort (48,49).
Venous access is typically obtained under ultra-
sound guidance. The site and extent of the occlu-
sion will guide the access site selection. In patients
with nonocclusive lesions, a single access site is
often sufficient, although dual access can provide
more options for imaging and better support for
device delivery. Many operators prefer the femoral
approach for convenience (49). In patients with total
SVC occlusion, additional access is advised, for
successful recanalization. Imaging directly from
previous allows for better assessment of lesion
length and facilitates crossing the lesion with the
guidewire (44). Options for access cephalad to the
SVC include the basilic, brachial, axillary, or internal
jugular veins. In patients with SVC stenosis or
TABLE 4 Detailed Characteristics of Studies Reporting Outcomes of SVC Syndrome After Endovascular Stent Placement
FirstAuthor(Ref.#) StentUsed n
Technical
Success (%) Restenosis
Recurrence of
SVC Syndrome
Kee et al. (31) Palmaz; Wallstent; Gianturco Z-stent 59 95 5 (8.5) 2 (3.3)
Fagedet et al. (32) Wallstent; Memotherm; SMART 163 84 16 (9.8) 32 (19.6)
de Gregoria Ariza et al. (33) Wallstent; Palmaz 82 100 7 (8.5) 1 (1.2)
Nagata et al. (34) Wallstent 71 100 8 (11.3) 8 (11.3)
Lanciego et al. (25) Wallstent 149 100 20 (13.4) 20 (13.4)
Dinkel et al. (35) Wallstent 84 99 19 (22.6) 8 (9.5)
Urruticoechea et al. (36) Wallstent; Memotherm 52 100 9 (17.3) 5 (9.6)
Nicholson et al. (37) Wallstent 76 100 9 (11.8) 9 (11.8)
Barshes et al. (38) Wallstent; Palmaz 56 100 4 (7.1) 6 (10.7)
Breault et al. (39) Wallstent 44 98 13 (29.5) 9 (20.5)
Maleux et al. (40) Zilver 78 99 8 (10.3) 8 (10.3)
Mokry et al. (41) Sinus XL stent 23 97 1 (4.3) 1 (4.3)
Gwon et al. (42) Covered ePTFE vs. uncovered 73 99 12 (16.4) 12 (16.4)
Total 1,010 98.6 131 (11.9) 121 (10.5)
Values are n (%), unless otherwise indicated.
ePTFE ¼expanded polytetrafluoroethylene; SVC ¼superior vena cava.
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occlusion with extension into the brachiocephalic or
subclavian vein, access from the ipsilateral brachial
or basilic vein facilitates successful traversal of the
lesion. Venography enhanced with digital subtrac-
tion, along with a breath-hold, provides
optimal visualization. In patients with total occlu-
sion, contrast administration in both arms may be
necessary, in order to identify direction of
flow, collateral veins, and presence of thrombus
(44,48).
CDT FOR THROMBOTIC LESIONS. CDT can be per-
formed from either upper extremity or lower ex-
tremity. The thrombotic occlusion is typically crossed
with a 0.035-inch hydrophilic guidewire supported
by a 4-F or 5-F diagnostic catheter; smaller caliber
guidewires can also be useful, depending upon the
individual anatomy. Once traversed, a venogram is
obtained to confirm intraluminal position distal to
the occlusion. A thrombolytic infusion catheter with
infusion length tailored to the occlusion span (usu-
ally between 12 cm and 30 cm) is then placed within
the thrombus and CDT initiated using well-
established thrombolytic agents (e.g., tissue plas-
minogen activator at a typical rate of 0.01 mg/kg/h
over 12 to 24 h; TNK and Retevase as alternatives are
also available). The patient is closely monitored,
and repeat venography is performed to assess for
residual thrombus, which can be treated with
FIGURE 4 Superior Vena Cava Obstruction From Catheter-Related Thrombosis
(A) A 29-year-old with congenital heart block status post permanent pacemaker presented with superior vena cava obstruction; coronal
computed tomography of the prominent azygos vein (red arrow) with right-sided collateral veins. (B) Sagittal view of prominent azogyos vein
with venous collaterals (red arrows).(C) Three-dimensional reconstructed images highlighting the azygos vein with various collateral veins.
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additional mechanical thrombectomy. This is fol-
lowedbyangioplastyandstentingoftheresidual
stenosis. Most patients with thrombotic occlusion are
initiated on systemic anticoagulation after revascu-
larization (50).
RECANALIZATION OF SVC CHRONIC TOTAL OCCLUSION.
Once the optimal imaging angles and length of oc-
clusion is defined, then a guidewire is used to cross
the lesion. Judicious use of multiple access sites
allows the operator to direct the guidewire with
greater accuracy. Multiple different guidewires and
techniques can be employed. Many operators will
first attempt crossing with a hydrophilic wire (e.g.,
glide wire, angled or straight, stiff, or floppy), sup-
ported by diagnostic catheter. If the lesion cannot be
crossed easily, more aggressive (e.g., “total occlusion-
type”) guidewires and crossing devices can be uti-
lized. Mechanical devices (e.g., Frontrunner [Cordis,
CENTRAL ILLUSTRATION Proposed Management Algorithm of SVC Syndrome Based on Severity
of Symptoms and Common Etiologies
Azizi, A.H. et al. J Am Coll Cardiol Intv. 2020;13(24):2896–910.
ABCs ¼airway, breathing, and circulation; BCV¼brachiocephalic vein; CXR ¼chest x-ray; CDT ¼catheter-directed thrombolysis;
ET ¼endovascular therapy; RT ¼radiation therapy; SCLC ¼small cell lung cancer; SVC ¼superior vena cava.
JACC: CARDIOVASCULAR INTERVENTIONS VOL. 13, NO. 24, 2020 Azizi et al.
DECEMBER 28, 2020:2896–910 SVC Syndrome
2905
MiamiLakes,Florida]),radiofrequencyenergy,laser
irradiation, or sharp recanalization with hydrophilic
wires can sometimes be utilized to initiate a track;
however,thesemustbeemployedwithextreme
caution, as rupture or perforation of the SVC is a
catastrophic complication (see the following). Once
the lesion is crossed successfully, pre-dilation with an
undersized balloon (2 to 4 mm) of the stenosis is
recommended. This is followed with sequential dila-
tion with increasing balloon diameters, assessing the
response of the vessel and the clinical reaction of the
patient (e.g., pain) with each dilation. Pre-dilation
facilitates stent deployment and expansion, but
oversizing the balloons to >16 mm has been associ-
ated with increased risk of SVC rupture, dysrhyth-
mias, pericardial tamponade, or cardiac arrest (32,49).
STENT SELECTION AND PLACEMENT. Balloon an-
gioplasty alone, without adjunct treatment, can pro-
duce rapid restoration of flow and relief of symptoms;
however, significant recoil and early restenosis and
reocclusion are common, owing to extrinsic
compression and the fibroelastic nature of the peri-
vascular tissue. Accordingly, contemporary recanali-
zation of SVC occlusion includes stent placement in a
majority of cases (50).Thechoiceofstentdependson
many factors including severity, length, and tortu-
osity of the SVC obstruction, and resistance to dila-
tion (Table 5)(51).
Pre-operative data from CT can be very useful to
choose the size and length of stents (1,35). Stent op-
tions include balloon expandable versus self-
expanding stents, and noncovered versus covered
stents. The most commonly used stents are the
balloon expandable stents (e.g., Palmaz stent [Cor-
dis]), self-expanding Wallstents (Boston Scientific,
Natick, Massachusetts) and Gianturco Z-stent (Cook
Medical, Bloomington, Indiana). Balloon-expandable
stents enable precise placement with reduced the
incidence of migration in addition to higher radial
TABLE 5 Venous Stents, Types, Descriptions, Benefits, and Drawbacks
Type of Stent Description Benefits Drawbacks
Wallstent
endoprosthesis
Elgiloy braided construction self-expanding
Available in large diameters
Well studied and widely used
Provides stent flexibility and efficacious in long
stenosis
Flexible and undergoes shortening after
deployment leading to decreased deployment
accuracy
Prone to migration
Poor radial strength
Palmaz stent Balloon-expandable stent High radial force useful in lesions with recoil
Allows staged dilatation of stenosis to larger
diameter
Lower propensity for stent migration
Relatively incompressible, which can lead to stent
fracture and reocclusion
Gianturco Z-stent 25–30 mm in diameter Preferable for isolated SVC stenosis
Small circumferential hooks prevent migration
Allows precise positioning
Rigid, making placement into tortuous vessels
difficult
Open-cell design and low contact with vessel wall
may not inhibit tumor ingrowth as effectively as
other stents do
Given diameter, requires wide bore sheath (up to
16-F) to deploy
Zilver Vena venous
stent
Open-cell design
Available in 14–16 mm diameters and 60–
140 mm lengths
Created for venous system
Offers flexibility and minimal foreshortening
Limited data
Venovo stent Open-cell design
8-F to 10-F platform and comes in 10–20 mm
diameters and 40–160 mm lengths
Created for venous system
Evidence has shown high patency rates in
iliofemoral venous obstruction
Has not been widely used for SVC syndrome and
data lacking
VICI stent Available in 12–16 mm diameters and 60–
140 mm lengths
Very good radial force Limited data
Viatorr stent Endoprosthesis designed for TIPS to treat
portal hypertension
Longest stent is 10 cm
Combination of bare stent and covered stent
Conformable to tortuous anatomy
Not widely used in SVC syndrome
Long-term patency of bare section of stent outside
of TIPS has not been studied
Viabahn VBX stent-
graft
Balloon-expandable covered endoprosthesis
Composed of independent
316 L stainless steel ring elements that are
connected via a heparinized fluoropolymer
graft material
Minimum slippage
Only covered stent lacking longitudinal struts for
ring linkage allowing maximal flexibility while
maintaining excellent radial strength
Food and Drug Administration approved for iliac
artery stenosis
Limited data for SVC syndrome
Sources: Shamimi-Noori and Clark TWI (52), Ganeshan et al. (49).
SVC ¼superior vena cava; TIPS ¼transjugular intrahepatic portosystemic shunt.
Azizi et al.JACC: CARDIOVASCULAR INTERVENTIONS VOL. 13, NO. 24, 2020
SVC Syndrome DECEMBER 28, 2020:2896–910
2906
force to overcome recoil. Self-expandable stents
conform better to the vessel wall of the vein and are
resistant to “two-point compression.”This allows for
volume changes in the patient’sthoracicspaceand
vessel diameter caused by respirations (52). To
ensure lesion coverage, use of longer self-expanding
stents is often required, which can lead to jailing of
the contralateral brachiocephalic vein (53). Many
operators currently prefer to use covered stents, such
as the Gore Viabahn (Gore, Newark, Delaware) and
iCast (Atrium, Dallas, Texas), based on reports of
better 12-month patency as compared with uncov-
ered stents. (54). Covered stents can prevent cata-
strophic extravasation in the event of SVC disruption
or frank rupture; however, care should be exercised
to avoid excluding collateral veins when using
covered stents. To date, a difference in clinical suc-
cess rates and overall patient mortality has not yet
been demonstrated between various types of stents
(42,55).
The Food and Drug Administration recently
approved dedicated venous stents (Venovo [Bard,
Minneapolis, Minnesota] and Vici [Boston Scientific]).
The VIRTUS (Safety and Efficacy of the Veniti
ViciVenous Stent System when Used to Treat Clin-
ically Significant Chronic Non-malignant Obstruction
of the Iliofemoral Venous Segment) and VERNAC-
ULAR (The BARDVENOVO Venous Stent Study –A
Prospective, Non-Randomized, Multi-Center, Single-
Arm Study of the Treatment of Iliofemoral Occlusive
Disease –an Assessment for Effectiveness and Safety)
trials showed excellent patency rates of these dedi-
cated venous stents in iliofemoral venous obstruction
(56,57). Data regarding the use of these new stents in
SVC syndrome are not yet available. Finally, use of a
hybrid stent-graft with a covered central portion and
uncovered ends, designed for transjugular intra-
hepatic portosystemic shunt placement (Viatorr
stent-graft [Gore, Flagstaff, Arizona]) has been used
successfully for SVC syndrome (58).
EXTENSION OF SVC OBSTRUCTION INTO BRACHIOCEPHALIC
VEINS. When SVC obstruction occurs with bilateral
brachiocephalic vein involvement, relieving the
obstruction in 1 of the occluded brachiocephalic veins
often suffices for symptom resolution. Dinkel et al.
(35) showed that recanalization and stenting of 1
instead of both brachiocephalic veins was associated
with lower rates of complications and stent throm-
bosis. Most operators avoid bilateral kissing stenting
if the SVC diameter is <15 mm.
Under certain circumstances, such as when the
occlusion involves the confluence of the brachioce-
phalic veins, reconstruction of the SVC bifurcation
may be appropriate. Typically, this reconstruction is
performed using “kissing stents,”also known as the
“double barrel”approach, by extending stents from
the SVC into the right and left brachiocephalic veins.
Depending on the individual anatomy, the “pant legs
approach”may be warranted, in which a separate
stent is deployed in the SVC proper, prior to placing
the 2 kissing stents into each of the brachiocephalic
branches. The benefit of having an SVC scaffold into
which the brachiocephalic stents feed may be a more
stable and durable reconstruction, although data are
not available yet to support this. During reconstruc-
tion of the SVC bifurcation, if self-expanding stents
are utilized as the limbs extending from the SVC into
the brachiocephalic veins, deployment of balloon-
expandable stents inside the self-expanding devices
can be performed to enhance the radial strength,
provide additional coverage, and ensure maximum
lumen area. To date, this has been done most
commonly with Palmaz stents within Wallstents, but
double-thickness stenting may also be performed
with newer devices. From a clinical perspective,
reconstruction of the SVC bifurcation with the
double-barrel approach creates natural bilateral con-
duits for future hardware, including catheters and
pacemakers or defibrillator leads.
Less commonly used is the “Y approach,”in
which one stent is passed through the wall of
another stent, then deployed to form a Y shape
(59,60). In contrast to the parallel method, the Y
method has lesser chances of stent migration and
creates a single, larger lumen in the SVC, which may
decrease the incidence of subsequent occlusions.
However, there is potential liability of passing one
stent through the wall of another, which can weaken
the integrity of the stent itself, leading to fracture
and dislocation (59).
ANTITHROMBOTIC THE RAPY AFTER ENDOVASCULA R
REVASCULARIZAT ION. Data guiding decision making
regarding intraprocedural and post-revascularization
pharmacologic therapy are also lacking, and practices
vary. In general, anticoagulation should be held, or the
patient “bridged,”immediately prior to the procedure.
Initial bolus with low-dose heparin may be prudent
untilsafeaccessisobtainedinallvenoussites,the
lesion is crossed, and intraluminal positioning is
confirmed.Atthatpoint,fulldoseheparinisadmin-
istered to achieve a therapeutic activated clotting
time (ACT). A brief period of triple therapy (anti-
coagulation, low-dose aspirin, and a thienopyridine)
may be considered in patients with thrombotic occlu-
sion if they are not too high a bleeding risk. In patients
with nonthrombotic obstruction, dual antiplatelet
JACC: CARDIOVASCULAR INTERVENTIONS VOL. 13, NO. 24, 2020 Azizi et al.
DECEMBER 28, 2020:2896–910 SVC Syndrome
2907
therapy with aspirin and a thienopyridine is
commonly administered for a month or longer,
although the evidence supporting this is lacking
to date.
COMPLICATIONS OF ET. Complications associated
with SVC revascularization are acceptably low and are
mitigated by operator experience and caution. Minor
complications are very infrequent and include he-
matoma and local infection at the puncture site
(3.2%). Major complications include pericardial tam-
ponade, SVC rupture, stent migration, in-stent
restenosis, pulmonary edema, major bleeding, pul-
monary embolism, and cardiac injury; these can be
catastrophic, even fatal. The cumulative incidence of
these complications is <8% (44). In patients with
malignant SVC syndrome, stent placement carries a
procedural mortality rate of 2% (55).
Pericardial tamponade is arare(0.1%to1.8%)but
dreaded and potentially fatal complication, particu-
larly when balloon sizes <16 mm are used (44). Pa-
tients who have undergone recent radiation may have
an increased risk of SVC rupture and extra caution
should be taken in these patients (1). Facilities offer-
ing this intervention should have availability of
emergency pericardiocentesis equipment and
personnel (23,61).
SVC rupture or extravasation outside the pericar-
dium can be equally catastrophic, resulting in blood
loss into the chest cavity or chest wall with precipi-
tous hypotension, pulmonary compromise, and
death. Interventionalists and staff must be prepared
to respond. Maintaining wire position across the
interventional site is critical, in order to deliver bal-
loons to immediately tamponade the site of disrup-
tion and curtail the blood loss. Emergency surgical
backup should be summoned. Covered stents or
stent-grafts can be used to “repair”or close the site of
extravasation.
Stent migration can occur as a result of stent
shortening, or if the stent is undersized and can be
managed with endovascular techniques such as
directly snaring the stent or balloon-guidewire–
assisted snaring (44). SVC stent occlusion is a
delayed complication and in patients with malig-
nancy is due to inward growth of tumor or extrinsic
compression from the mass. Alternatively, reocclu-
sion can be caused by intimal hyperplasia or
thrombus formation (23). Repeat stenting can often
be effective in treating restenosis and even reoc-
clusion (32). Pulmonary edema is rare and caused by
a precipitous increase in venous return after a
stenting. Patients with underlying poor cardiac
reserve are more susceptible and should be treated
with diuretic agents (44,62). The post-procedure
surveillance includes a close clinical follow-up usu-
ally every 3 months and a repeat venography if
symptoms recur. Patients are advised to call imme-
diately if symptoms recur (23).
FUTURE OUTLOOK
The management of SVC syndrome has evolved over
the last 3 decades as interventional techniques have
progressed. Nevertheless, there is paucity of robust
data and a lack of formal societal guidelines or expert
consensus available for clinicians treating patients
with SVC obstruction. Standardized methods are
needed to categorize SVC syndrome, grade the
severity, and guide therapy. The availability of dedi-
cated venous and covered stents provides more op-
tions for treating these patients; the role of these new
devices should be clarified with more experience and
evidence.
The optimal anticoagulation and antiplatelet regi-
mens for patients with SVC syndrome deserves
more attention and will only be established with more
data collection. Currently, for cases of SVC obstruc-
tion with significant thrombosis, systemic anti-
coagulation is the standard of care, both before and
after revascularization. However, in the absence of
notable thrombosis, the utility of long-term anti-
coagulation or antithrombotic therapy has not been
well established. Some have opined that systemic
anticoagulation can decrease the supposed risk of
in-stent thrombosis (34,35) while others have pro-
posed that antiplatelet therapy alone is sufficient and
appropriate (25).
CONCLUSIONS
SVC syndrome affects a small proportion of patients,
but its effects can be devastating. Malignancy re-
mains the commonest cause of SVC obstruction,
although there is an increased incidence of nonma-
lignant SVC syndrome in recent years because of the
proliferation of devices and catheter placement.
First-line treatment of SVC syndrome has evolved
from RT to ET. While treatment options have
expanded and new modalities and devices potentially
offer more safe and durable solutions, there is a need
for standardization of treatment strategies. In the
current paper, we propose a classification system that
integrates severity of symptoms and location of
obstruction to guide subsequent management. Use of
this novel paradigm will enable consistency in
Azizi et al.JACC: CARDIOVASCULAR INTERVENTIONS VOL. 13, NO. 24, 2020
SVC Syndrome DECEMBER 28, 2020:2896–910
2908
reporting and development of an expanded evidence
base regarding management of SVC obstruction.
AUTHOR DISCLOSURES
Dr. Rosenfield has served as a consultant for Abbott, Access Vascular,
Accolade, Capture Vascular, Endospan, Magneto, Micell, Shockwave,
Silk Road, Surmodics, Thrombolex, and Valcare; owns equity interest
in Access Vascular, Contego, Embolitech, Eximo, MD Insider, Jana-
care, PQ Bypass, and Primacea; and has received research or
fellowship support from National Institutes of Health. Dr. Bashir
owns equity interest in Thrombolex Inc. All other authors have re-
ported that they have no relationships relevant to the contents of this
paper to disclose.
ADDRESS FOR CORRESPONDENCE: Dr. Riyaz Bashir,
Temple University Hospital, Division of Cardiovas-
cular Diseases, 3401 North Broad Street (9PP), Phila-
delphia, Pennsylvania 19140, USA. E-mail: riyaz.
bashir@tuhs.temple.edu.
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KEY WORDS catheter-directed
thrombolysis, endovascular therapy,
superiorvenacavaobstruction
APPENDIX For a supplemental figure and
tables, please see the online version of this
paper.
Azizi et al.JACC: CARDIOVASCULAR INTERVENTIONS VOL. 13, NO. 24, 2020
SVC Syndrome DECEMBER 28, 2020:2896–910
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