STUDENT PROJECT

CARDIOVASCULAR SYSTEM AND DISORDER

SUBCLAVIAN STEAL SYNDROME

SGD B10
Ditulis Oleh:
Olivia Vanya Wardoyo 1602511044
Jason Jonathan 1602511093
Putu Putri Titamia Saraswati 1602511097
Randy Martianus 1602511098
Kadek Intan Jelita 1602511107
Prisillia Brigitta 1602511113
I Wayan Agus Surya Pradnyana 1602511149
Gede Ari Mahendra Mardaningrat 1602511174
Luh Gede Sarita Giovanni 1602511198
Dhaanavendharan Puvanthiran 1602511240
Eswariy Chandramogan 1602511246

FAKULTAS KEDOKTERAN
UNIVERSITAS UDAYANA
2019/2020
 CHAPTER I
INTRODUCTION
Subclavian ‘steal’ phenomenon is a function of the proximal subclavian artery
steno-occlusive disease, with subsequent retrograde blood flow in the ipsilateral
vertebral artery. A syndrome of symptoms relating to arterial insufficiency in a branch
of the subclavian artery stemming from flow reversal, attributable to occlusive disease
in the subclavian artery proximal to that branch that is usually atherosclerotic in
cause.[1]
The symptoms from the compromised vertebrobasilar and brachial blood flows
constitute the subclavian steal syndrome, and include paroxysmal vertigo, drop attacks
and/or arm claudication. Once thought to be rare, the emergence of new imaging
techniques has drastically improved its diagnosis and prevalence. The syndrome,
however, remains characteristically asymptomatic and solely poses no serious danger
to the brain. Recent studies have shown a linear correlation between increasing arm
blood pressure difference with the occurrence of symptoms. Atherosclerosis of the
subclavian artery remains the most common cause.[1]
Subclavian steal phenomenon quite rare, The Joint Study of Extracranial
Arterial Occlusion yielded 2.5% incidence (168/6534), with only 5.3% of these patients
experiencing neurological symptoms. The most recent large scale prevalence study.
Corroborates these previous findings, with 5.4% of Subclavian Steal Syndrome cases
found after a series of 7881 ultrasound exams of the extracranial neck vessels.
The acquired subclavian steal phenomenon is mostly left-sided reporting 82.3%
of their cases. Most authors estimate the ratio between the left-and right-side subclavian
steal to be 4:1. Males are affected by the atherosclerotic subclavian steal phenomenon
more than females by a ratio of about 2:1. Patients above the age of 50 are also more
likely to present with subclavian steal syndrome, most likely due to increased
atherosclerosis in this age group.[1]
Diagnostic tool for Subclavian steal syndrome usually using Doppler
ultrasound, it is a useful screening tool, but the diagnosis must be confirmed by CT or
MR angiography. Conservative treatment is the initial best therapy for this syndrome,
with surgery reserved for refractory symptomatic cases. Percutaneous angioplasty and
stenting, rather than bypass grafts of the subclavian artery, is the widely favored
surgical approach. Nevertheless, large, prospective, randomized, controlled trials are
needed to compare the long-term patency rates between the endovascular and open
surgical techniques.[2]
Some studies said the use of arm blood pressure as a predictor of the severity of
the syndrome.[21] Then, delayed or decreases amplitude pulses in the affected side, the
skin and nails in the affected side might changes due to arterial insufficiency.[20]
Significant ischemia of the arm is rare, even in patients who have complete occlusion
of the proximal subclavian artery.[21]
Treatment of subclavian steal syndrome is usually reserved for symptomatic
patients with associated significant carotid artery occlusion or for prophylactic
treatment for stroke by treating the carotid artery stenosis. Most patients with proximal
subclavian stenosis and reversed vertebral blood flow experience no cerebrovascular
symptoms, some patients may be symptomatic if there is additional intracranial or
extracranial vascular obstruction.[3]
Because the Subclavian Steal Syndrome quite rare but it may cause a significant
ischemia of the arm, in this student project we aim to present the epidemiology,
etiology, pathophysiology and common variants of the subclavian steal syndrome to
let our readers learn about the harm of the disease. We also present the current
recommendations on the best approach to diagnosis and treatment of this syndrome
based on recent literature review.
 CHAPTER II
THEORY AND DISCUSION

2.1 Definition of Subclavian Steal Syndrome

Subclavian steal syndrome is a rare condition causing syncope or neurological
deficits when the blood supply to the affected arm is increased through exercise.
Subclavian steal is secondary to a proximal stenosing lesion or occlusion in the
subclavian artery, typically on the left. Stenosis in the subclavian artery is commonly
caused by atherosclerosis, however less common causes of the condition include
vasculitis, thoracic outlet syndrome such as cervical rib or complications following
aortic coarctation repair and which can lead to manifestations of cerebral vascular
insufficiency.[4]
The syndrome presents several anatomic and clinical variants, which commonly
characterised by the diversion of arterial flow from the cerebral area to the upper limb
due to reversed flow in the vertebral artery, ipsilateral to the stenosis or occlusion. The
diversion is more important in cases of severe stenosis or subclavian occlusion, where
steal is complete (occurring in all stage of the cardiac cycle), while it is quantitatively
less significant in moderate stenosis, where blood steal is intermittent (occurring only
during the cardiac systole) and in mild stenosis where blood steal is latent (occurring
only after prolonged post-ischemic reactive hyperaemia manoeuvres of the upper
limb).[5]
2.2 Epidemiology of Subclavian Steal Syndrome
Most literatures report the prevalence of subclavian steal syndrome as between 0.6%
to 6.4%. The Joint Study of Extracranial Arterial Occlusion by Fields et al. yielded
2.5% incidence (168/6534), with only 5.3% of these patients experiencing neurological
symptoms. The most recent large-scale prevalence study by Labropoulos et al.
corroborates these previous findings, with 5.4% of SSS cases found after a series of
7881 ultrasound exams of the extracranial neck vessels.[6]
The acquired subclavian steal phenomenon is mostly left-sided with Labropoulos et
al. reporting 82.3% of their cases. Most authors estimate the ratio between the left-and
right-side subclavian steal to be 4:1. The proposed explanation is that the acute angle
of origin of the left subclavian artery increases flow turbulence and accelerates
atherosclerosis at the subclavian-aortic junction. The left subclavian artery is also the
most commonly affected artery in Takayasu arteritis (85% of cases), a rare cause of the
subclavian steal syndrome.[7]
Males are affected by the atherosclerotic subclavian steal phenomenon more than
females by a ratio of about 2:1. Patients above the age of 50 are also more likely to
present with subclavian steal syndrome, most likely due to increased atherosclerosis in
this age group. The steal from Takayasu’s disease, however, presents far earlier (30
years in 90% of cases) and more commonly in the female gender A study by Watts
and his colleagues ] reported that in the Far East, Takayasu’s arteritis was the cause in
36% (9/25) of patients requiring surgery to correct the subclavian steal syndrome, with
the remaining cases stemming from atherosclerosis.[8]

2.3 Etiology of Subclavian Steal Syndrome

A subclavian steal syndrome occur when there is a stenosis in the subclavian artery.
The most common cause of subclavian stenosis is atherosclerosis. other possible causes
of subclavian stenosis are large artery vasculitis, thoracic outlet syndrome, and stenosis
after surgical repair of aortic coarctation or tetralogy of Fallot (with a Blalock-Taussig
shunt). Rare causes of subclavian stenosis include aortic dissection, Takayasu’s
arteritis, external compression on the subclavian artery and anatomical anomalies such
as an isolated innominate artery, Congenital abnormalities, such as a right-sided aortic
arch with an isolated left subclavian artery.[9,10]
Rare cases of congenital subclavian steal caused by an anomalous right-sided aortic
arch, may result in a congenital left-sided steal by way of an isolation of the left
subclavian artery from the aorta.[11]

2.4 Classification of Subclavian Steal Syndrome

The subclavian steal syndrome is classified by the territory from which the blood is
“stolen”.[12] Those territories are classified as vertebral-vertebral, carotid-basilar,
external carotid-basilar, or carotid- subclavian. The subclavian steal syndrome can also
be classified into the severity of hemodynamic disturbances in the vertebral artery.2
Then, severity is classified into three stages or grades, such as:[13]
1. Grade I (pre-subclavian steal) : the antegrade vertebral flow is reduced;
2. Grade II (intermittent/partial/latent) : alternating flow – antegrade flow in
the diastolic phase and retrograde flow in the systolic phase;
3. Stage III (permanent/advanced) : permanent retrograde vertebral flow.
A recent study said that the prevalence of a complete steal increases with higher arm
blood pressure differentials (PD).[14] The study has shown that an arm PD> 40 mmHg
was associated with significantly increased prevalence of partial and complete steal,
with a greater proportion of the latter.[14] All the patients under study who had no
identified steal on ultrasound had a PD<40 mmHg. This corroborates an earlier study
by Tan and his colleagues that showed a modest correlation between vertebral arterial
waveforms and the blood pressure differentials in patients with subclavian steal
syndrome.[15]

2.5 Pathophysiology Classification of Subclavian Steal Syndrome

Subclavian steal phenomenon often involves high-grade stenosis and/or occlusion
of the subclavian artery, leading to a decrease in the SA pressure distal to the lesion. If
the stenosis is severe and the affected arm is exerted, this pressure drop can cause the
blood flow in the ipsilateral vertebral artery to reverse, stealing the blood from the
unaffected SA via the contralateral vertebral artery in order to ensure adequate blood
supply to the involved arm. Vigorous exercise of the arm and a sudden sharp turning
of the head in the direction of the affected side have so far been shown to provoke the
symptoms.[16,17]

Figure 1. Schematic drawing showing a left-sided subclavian steal syndrome.

The pathophysiologic mechanism behind the subclavian steal via the vertebral artery
is similar to the one involving coronary arteries, except that different vessels are
involved. The coronary subclavian steal syndrome (CSSS) has been defined as the
reversal of flow in a previously constructed internal mammary artery (most often left
internal mammary artery) and left coronary artery bypass graft, leading to myocardial
ischemia.[18] The proximal subclavian artery stenosis in this scenario causes reversed
flow in the ipsilateral IMA coronary artery graft. The potential red flags for CSSS
include refractory unstable angina in a patient with a history of CABG and/or impaired
weaning of such patients after cardiopulmonary bypass. More recently, cardiac arrest
from ventricular fibrillation has been reported.[19]

Figure 2. Schematic diagram showing the retrograde from the left coronary
artery through the mammary artery bypass grafts in a patient with left subclavian
artery Stenosis

2.6 Clinical Manifestation of Subclavian Steal Syndrome

Generally, most patients with subclavian steal syndrome (SSS) or subclavian
stenosis are usually asymptomatic. The symptoms will be appeared if the collateral
blood supply from the vertebrobasilar circulation cannot bear increased demand, such
as during exercise or in patient with an arteriovenous fistula. In some case related to
subclavian stenosis, arm caudation is the most common symptoms, induced by exercise
or fatigue condition.[20] Ischemia to the hand often manifests claudication, paresthesias
(coolness in the arm).[21] Vertebrobasilar insufficiency may also manifest as dizziness,
diplopia, nystagmus, tinnitus, or hearing loss.[20,21]
In physical examination findings, a different blood pressure between both upper
extremities is founded (>15 mmHg).[20] Some studies said the use of arm blood pressure
as a predictor of the severity of the syndrome.[21] Then, delayed or decreases amplitude
pulses in the affected side, the skin and nails in the affected side might changes due to
arterial insufficiency.[20] Significant ischemia of the arm is rare, even in patients who
have complete occlusion of the proximal subclavian artery.[21]
2.7 Diagnosis and Differential Diagnosis of Subclavian Steal Syndrome
The subclavian steal syndrome is a rare but important cause of syncope. Since
recognition of this syndrome can lead to successful treatment, this emphasizes the need
for a high index of suspicion in patients with a suggestive history, risk factors and
physical findings, such as symptoms of vertebrobasilar insufficiency.[22]
Subclavian stenosis should be suspected in any patient with vertebrobasilar territory
neurological symptoms, arm claudication, or coronary ischemia where the IMA has
been used for coronary artery bypass graft surgery. Physical examination findings
suggestive of subclavian stenosis include a discrepancy of >15 mmHg in blood
pressure readings taken in both upper extremities, delayed or decreased amplitude
pulses in the affected side, and a bruit in the supraclavicular fossa.[23]
The syndrome results from occlusion of the proximal subclavian artery and the
development of retrograde flow to the subclavian artery from the vertebral artery. The
decreased blood flow to the posterior brain and upper extremity on the affected side
results in a range of symptoms. Vertebrobasilar insufficiency may produce light-
headedness, dizziness, ataxia, vertigo, visual disturbances, motor deficits, confusion,
focal seizures, aphasia, headaches or syncope. Reduced blood flow to the arm causes
symptoms such as weakness, coldness, paresthesias or arm claudication after exercise.
Arm symptoms are generally less frequent and occur later than the vertebrobasilar
symptoms. Classic signs in subclavian steal syndrome include a difference in brachial
artery pressures of 20 mmHg or greater, weak or absent radial pulse, and
supraclavicular bruit on the ipsilateral side.[23]
A variety of noninvasive imaging modalities can be selectively used to diagnose
subclavian stenosis when a steal phenomenon is suspected. Continuous wave Doppler
and duplex ultrasonography are readily accessible, inexpensive, and accurate when
performed by an experienced operator. The diagnosis may be confirmed by carotid
duplex ultrasonography and magnetic resonance angiography, or arch aortography.[23]
Bilateral blood pressure measurement is the simplest and most cost-efficient method
for screening of SAS, but can miss patients who have equal bilateral subclavian artery
stenosis. Subclavian artery angiography remains the gold standard and can be
performed during coronary angiography. A pattern of flow in the result of angiography
showing a severe proximal left subclavian stenosis, with retrograde flow in the left
vertebral artery, and the resulting clinical symptoms, is known as the subclavian steal
syndrome [24]
2.8 Treatment and Management of Subclavian Steal Syndrome
1. Pharmacotherapy
It is known that no pharmacotherapy can effectively treat subclavian steal syndrome.
However, pharmacotherapy is used to reduce morbidity and prevent further
complication. This pharmacotherapy includes antiplatelet agents. Antiplatelet agents
inhibit platelet aggregation and patients should be treated for life time therefore
reducing the risk of myocardial infarction, stroke, and other vascular disease that can
cause mortality. The medication includes aspirin, clopidogrel, and ticlopidine.[25,2 6]
2. Surgical
Surgical revascularisation consists of bypass in the form of carotid-subclavian
bypass, carotid transposition, and axillo-axillary bypass. Bypass or transposition is the
gold standard treatment for patients with subclavian steal syndrome. Before the surgery
procedure, arch aortography must be done to visualize the carotid and to make sure the
proximal common carotid and distal subclavian arteries are free of disease. Post-
operative, patient should be monitored for neurologic deficit.[25]
Complication of surgical procedures can be classified into local and cerebral. Local
complication will happen if there is any injury to the structures encountered during the
surgery. Cerebral complication is related to brain ischemic symptoms and can lead to
post-operative strokes.[25,26]
3. Endovascular Treatment
Endovascular recanalization and stenting are done when the patients are associated
with plaque emboli and bleeding. A guide wire must first be placed across the lesion
before placing the balloon-expandable stents. This procedure improve perfusion to the
arm and successfully treat subclavian steal syndrome. Post procedure, patients should
be monitored to ensure there is no bleeding or hematoma.[26]

2.9 Prognosis and Complication of Subclavian Steal Syndrome