Imaging Teaching Case

Complications of Vascular Access: Superior Vena

Cava Syndrome
Anil K. Agarwal, MD,1 Hooman Khabiri, MD,2 and Nabil J. Haddad, MD 1

Stenosis or occlusion of central veins in hemodialysis patients is common, especially with previous intra-
vascular catheter or device use. Superior vena cava (SVC) obstruction is emerging as a frequent chronic
complication of central vein cannulation that not only jeopardizes the availability of vascular access for
hemodialysis, but can become a life-threatening emergency. Clinical features of SVC syndrome can be subtle
or dramatic, including facial swelling and shortness of breath, which require expeditious attention and inter-
vention. The approach to SVC syndrome involves judicious use of imaging techniques to define the cause and
location. Early management with endovascular intervention with angioplasty and stent placement is the usual
first choice. The occlusion can often be recanalized using new techniques such as radiofrequency wire and
then salvaged with stents, providing prompt resolution of symptoms. Limitations to interventions include
requirement of cutting-edge equipment, expertise, expense, and the usually temporary nature of the resolu-
tion. Surgery is considered the treatment of last resort for refractory cases. SVC syndrome can be prevented
by minimizing catheter and intravascular device use through early recognition of patients with chronic kidney
disease, early referral for education about all choices for kidney replacement modalities, and early placement
of arteriovenous access prior to the onset of dialysis therapy.
Am J Kidney Dis. -(-):---. ª 2016 by the National Kidney Foundation, Inc.

INDEX WORDS: Superior vena cava (SVC); SVC syndrome; obstruction; central vein stenosis; occulsion;
dialysis catheter complications; tunneled dialysis catheter; vascular access for hemodialysis; radiofrequency
wire; angiography.

INTRODUCTION location and physical characteristics of stenosis or

In 2013, a total of 88.2% of all incident patients
with end-stage renal disease (ESRD) began renal
replacement therapy with hemodialysis (HD) in the
United States.1 Despite an increase in arteriovenous
(AV) fistula use at HD therapy initiation from 12% to
17.1% between 2005 and 2013, a majority of patients
initiating HD therapy in the United States do so with a
central venous catheter (CVC). In particular, in 2013,
the proportion of patients using a CVC at HD therapy
initiation was 80.2%, a value that has changed little
since 2005. Furthermore, in data from 2013, at 90
days after dialysis therapy initiation, 68.3% of HD
patients were still using a CVC. Catheters are asso-
ciated with acute complications of placement, as well
as chronic complications of venous stenosis or
occlusion. A recent systematic review of 62 cohort
studies concluded that CVCs were associated with the
highest risk for death, infection, and cardiovascular
events compared with other vascular access types and
that patients with a functional AV fistula had the
lowest risk.2
Superior vena cava (SVC) syndrome is the result of
stenosis or occlusion of the SVC or bilateral bra-
chiocephalic veins. The clinical diagnosis of SVC
syndrome is based largely on history and physical
examination. Appropriate imaging techniques are
important to confirm the diagnosis and rule out other
causes of SVC syndrome. Determining the exact
occlusion is crucial prior to intervention, and newer
technologies for intervention are available that can be
used in select cases.
We present the case of an HD patient with debili-
tating symptoms and signs of SVC syndrome. Clin-
ical diagnosis of SVC syndrome was obvious, but
treatment required extensive imaging and interven-
tion, which resulted in resolution of distressing
symptoms.
CASE REPORT
Clinical History and Initial Laboratory Data
A 45-year-old man with ESRD secondary to diabetes mellitus
and hypertension presented with 2 years of progressive right arm
and facial swelling that started shortly after the creation of a right
upper-arm AV graft (AVG). He experienced difficulty sleeping at
From the 1Division of Nephrology and 2Department of Radi-
ology, The Ohio State University Wexner Medical Center,
Columbus, OH.
Received May 27, 2016. Accepted in revised form August 21,
2016.
Address correspondence to Anil K. Agarwal, MD, Section of
Nephrology at University Hospital East, Interventional
Nephrology, The Ohio State University Wexner Medical Center,
395 W 12th Ave, Ground Flr, Columbus, OH 43210. E-mail: anil.
agarwal@osumc.edu
 2016 by the National Kidney Foundation, Inc.
0272-6386
http://dx.doi.org/10.1053/j.ajkd.2016.08.040

Am J Kidney Dis. 2016;-(-):--- 1


night and inability to dress himself due to the extensive head and
arm swelling. The AVG had been working well, with no episodes
of thrombosis. There was a history of multiple CVC placements on
both sides of the neck.
On physical examination, the patient had massive facial
swelling and periorbital edema causing his eyes to be almost shut.
There was massive right arm swelling, but no swelling of the left
arm. The right arm, forearm, and hand were substantially larger
than the left. Extensive venous collateralization was noted on the
left side of the chest. The right upper-arm AVG had a palpable
thrill.
Imaging Studies
Computed tomography (CT) venography of the chest demon-
strated a short-segment occlusion of the SVC between the
confluence of the brachiocephalic vein above and the azygous
venous inflow below (Fig 1). The SVC was of good caliber below
the occlusion. The left brachiocephalic vein was absent, consistent
with chronic occlusion. The right brachiocephalic vein was patent
and moderately enlarged. Massive collaterals throughout the chest,
shoulder, and mediastinum were demonstrated.
Diagnosis
The patient exhibited almost the entire spectrum of SVC syn-
drome, with imaging correlate of SVC occlusion involving the
segment between the confluence of the brachiocephalic vein and
the azygous vein inflow. An intervention was planned.
Clinical Follow-up
Endovascular recanalization was performed under general
anesthesia. The right upper arm and right femoral area were pre-
pared and the venous limb of the AVG was accessed. A 7F by
55-cm sheath was advanced over the wire. Venography through
the sheath confirmed the findings (Fig 2A). A multiloop snare
device device was introduced and opened just above the SVC
occlusion at the level of the brachiocephalic vein confluence. Then
a right common femoral vein was accessed and a 9F by 55-cm
sheath was placed. Venography was performed to better evaluate
Figure 1. Computed tomography angiogram shows the
occluded segment of the superior vena cava (SVC). Note a large
number of collateral veins over the entire upper body and partic-
ularly enlarged collaterals on the left in this patient with SVC and
left brachiocephalic vein occlusion.
2 Am J Kidney Dis. 2016;-(-):---
Agarwal, Khabiri, and Haddad
the SVC. A directional catheter and conventional hydrophilic wire
were unable to cross the SVC occlusion.
A radiofrequency Powerwire (Bayliss Medical) was then
introduced through the femoral sheath. Careful multiplane oblique
fluoroscopy was performed, spanning an arc of at least 90 to
ensure proper alignment of the radiofrequency wire located caudal
to the occlusion with the snare located cephalad to the occlusion
(Fig 2B). Careful pulse activation of the radiofrequency wire was
performed 5 times before the occluded segment was successfully
crossed. Direct fluoroscopic guidance was used to ensure
continued alignment of the wire in the direction of the snare. The
wire was snared and externalized via the right upper-extremity
sheath. Careful sequential angioplasty was performed up to
8 mm 3 4 cm with postangioplasty venography after each balloon
dilatation to ensure the absence of extravasation (Fig 2C). After
balloon dilatation to 20 mm, the right femoral sheath was
exchanged for a 12F by 45-cm sheath. A 24 3 60-mm Wallstent
(Boston Scientific Corp) was deployed in the newly recanalized
SVC and serially dilated to 22 mm. A poststent/angioplasty
venogram demonstrated a wide-open SVC (Fig 2D).
In the recovery area and during the ensuing few days, the patient
had shortness of breath and fluid overload thought to be due to the
establishment of high AV flow and mobilization of fluid from his
upper torso. He had immediate and continued improvement in his
facial and arm swelling beginning a day after the procedure and
continuing for several days. After several days of dialysis, he was
discharged from the hospital.
DISCUSSION
William Hunter first described SVC syndrome due
to a syphilitic aortic aneurysm in 1757.3 Malignancy
has been the predominant cause of SVC syndrome,
although with the emergence of central venous
devices, up to 40% of all causes and 71% of benign
causes of SVC syndrome have been attributed to
these.4 However, patients with dialysis-related SVC
syndrome are not as frequently symptomatic as those
with malignancy, and the prevalence of SVC syn-
drome in HD patients may be underestimated. In a
case series, HD patients had a slower onset of
symptoms and higher likelihood of complete SVC
obstruction (85%) than those with a chest neoplasm,
whose obstruction was more likely to be partial
(67%).5 Furthermore, the prognosis in HD patients
was nearly as bad as in those with malignancy. Within
2 years, 31% of the patients died; 60% of these were
symptomatic.
The SVC is the final common pathway for venous
drainage of the upper half of the body. The SVC is
formed by the confluence of bilateral brachiocephalic
veins and is w7 cm in length. It receives the azygous
vein prior to entering the pericardium that covers its
lower half. There are multiple mechanisms causing
SVC syndrome (Box 1). Malignancies usually cause
direct infiltration or compression of the SVC. In HD
patients, the obstruction occurs due to a combination
of stenosis and thrombosis. The pathophysiology can
be explained by Virchow’s triad, comprising vessel
injury, hypercoagulability, and stasis caused by an
intravascular device. Also, the turbulence due to an
Central Venous Catheter Induced Superior Vena Cava Syndrome

Figure 2. Endovascular recanalization. (A) Angiography shows occlusion of the superior vena cava (SVC) at the level of the conflu-
ence of the innominate veins and the presence of collateral vessels. (B) When crossing the lesion with radiofrequency wire, using a
snare to mark the position of the target vessel before crossing the lesion is a must. (C) Careful sequential angioplasty of the lesion
beginning with smaller balloons and gradually using larger balloons. Note the focal waist on the balloon marking the “shelf” of fibrosis.
(D) Placement of stent to recanalize the SVC. Note the absence of previously visualized collaterals.

intravascular device and increased flow from creation
of an AV access may incite inflammatory, thrombotic,
and fibrotic responses, resulting in neointimal hyper-
plasia, adhesive bands and stenosis, or occlusion of
the lumen.6 As SVC flow is compromised, collateral
flow develops via the azygous, intercostal, internal
mammary, and long thoracic veins, partially offsetting
the initial symptoms. Later, shortness of breath and
orthopnea, swelling of the face and arm, feeling of
fullness of the head and lightheadedness, visual
symptoms, chest pain, dysphagia, glottis edema,
hoarseness, cough, and pleural effusions occur. As in
our case, physical examination can show swelling of
the face and arms, periorbital edema, collateral vein
engorgement, cyanosis, papilledema, and altered
consciousness. Symptoms often worsen on lying
down or bending forward. Airway compromise can be
life-threatening.
Diagnosis of SVC syndrome is usually easy, but
should be differentiated from pulmonary disease,

Am J Kidney Dis. 2016;-(-):--- 3


Box 1. Causes of Superior Vena Cava Syndrome
 Extraluminal (compression)
 Tumors with or without invasion and thrombosis
of superior vena cava syndrome: bronchogenic
carcinoma, lymphoma, metastatic cancers
 Mediastinal processes: fibrosis, nonmalignant tumors
(eg, thymoma, cystic hygroma, teratoma)
 Vascular causes: aneurysms, arteriovenous fistulas,
pericardial disease
 Infections
 Intraluminal: Usually a result of intravascular devices
 Thrombosis
 Stenosis/occlusion
 Idiopathic
heart failure, pericardial tamponade, and cellulitis.
Imaging studies are crucial to determine the cause,
management, and prognosis. Duplex ultrasonography
is suboptimal in the evaluation of the SVC or central
veins due to interference by the bony thorax. Plain
radiographs and CT of the chest can reveal the pres-
ence of a malignancy or cardiopulmonary process.
Magnetic resonance imaging is superior in defining
soft-tissue processes, but gadolinium should be
avoided in patients with ESRD due to the risk for
nephrogenic systemic fibrosis. CT angiography is
very useful in localizing stenosis or occlusion of the
central veins and SVC. Finally, digital subtraction
angiography is required as the intervention is planned.
Management of SVC syndrome depends on the
cause and severity, with an approach similar to that
for management of central vein stenosis, but with
more urgency.7 In general, the head is to be elevated.
Dyspnea can be treated with oxygen. A thrombotic
occlusion can often be treated with catheter-directed
thrombolysis using alteplase or other thrombolytic
agents. An indwelling device usually needs removal,
though presence of a thrombus may require anti-
coagulation first.
For SVC stenosis or occlusion, endovascular inter-
vention is the primary choice because it is minimally
invasive. Angioplasty alone, though technically highly
successful, has poor long-term patency.8,9 This is
attributed to the higher recoil of central veins than
peripheral veins, as shown by intravascular ultraso-
nography.10 Results of bare-metal stent placement
have been variable, with limited short- and long-
term patency requiring frequent intervention.11,12
Recently, covered stents have shown better results in
the treatment of central vein stenosis.13,14 A relatively
new approach involves placement of a hybrid
graft-catheter device.15 The catheter portion of
the device bypasses the SVC stenosis. It is limited
by often tedious placement and the occurrence of
thrombosis or infection with similar frequency as
in AVGs.
4 Am J Kidney Dis. 2016;-(-):---
Agarwal, Khabiri, and Haddad
Recanalization of an occluded SVC, as in our
patient, requires more extensive endovascular inter-
vention. Sharp recanalization with a classic hydrophilic
wire followed by angioplasty and stent placement
involves using the back end of a wire or other sharp
object to cross an occluded segment. The disadvantage
is the relative lack of control of the operator over the
exact trajectory of the wire, which is dictated by the
angle of approach and amount of resistance offered by
the occluding lesion. This may force the wire into a
course that is not optimal for subsequent balloon dila-
tation because it is too peripheral within the occluded
vessel or even extravascular. Additionally, it is difficult
to change the course after a false passage has been
made. However, it is inexpensive, is readily available,
and could be useful for short-segment occlusions in
which the wire is already in a central path and is
meeting more resistant occlusion.
A new technology for recanalization uses radio-
frequency wire, which is a 0.035-inch exchange
length wire with an active end. When energized with
a radiofrequency pulse, it creates a small zone of burn
within the tissue and can facilitate crossing lesions
that would otherwise be impossible to cross. This
allows careful and controlled advancement of the wire
in short increments without the need for excessive
force. Operator control over the course of the wire is
significantly better than with sharp recanalization.
The tendency to follow a false passage is less prob-
lematic and the wire can still be guided in various
directions. Avoiding vessel perforation requires
careful fluoroscopic evaluation and interpretation of
the images to stay intravascular and recognize an
extravascular course if it happens. Extravascular
passage of the wire is unlikely to cause a major
complication, particularly because the recanalized
vein is not likely to bleed. If the wire enters and
crosses adjacent structures such as arteries, subse-
quent balloon dilatation can cause potentially signif-
icant complications.
The radiofrequency wire is best used to cross short-
segment occlusions when there are normal vessel
segments above and below the occlusion. A vascular
snare device on the other side of the occlusion acts as
a target to guide advancement of the radiofrequency
wire. The wire should be activated and advanced
only a few millimeters at a time to avoid adjacent
structures. There is a significant learning curve with
potential for complications unless strict recommen-
dations are followed. With the advent of computer-
ized guiding software, the technique might find
broader application. As in our case, it is also impor-
tant to observe the patient for symptoms of volume
overload after successful recanalization of the SVC.
Unless thoracotomy is considered necessary, sur-
gery is reserved for refractory cases only because of
Central Venous Catheter Induced Superior Vena Cava Syndrome
the frequent comorbid conditions in patients with
ESRD. The options include vein patch or graft repair
of the SVC or surgical bypass of the occlusion with
direct anastomosis to the proximal SVC or right
atrium.
In conclusion, we describe a patient on HD therapy
with an AVG, history of multiple CVCs, and gradual
onset of severe SVC syndrome. SVC syndrome was
diagnosed on presentation and studied with extensive
imaging techniques. With careful use of an endovas-
cular recanalization technique, there was resolution of
SVC occlusion and symptoms. Prevention of venous
stenosis by using the “catheter last” approach through
early education and planning for placement of an AV
access and use of other modalities of dialysis remains
the preferred approach in kidney patients.
ACKNOWLEDGEMENTS
Support: None.
Financial Disclosure: The authors declare that they have no
relevant financial interests.
Peer Review: Evaluated by 2 external peer reviewers, Feature
Editor Kalantor-Zaheh, Education Editor Gilbert, and Editor-in-
Chief Levey.
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