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Subclavian Steal Phenomenon

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Synonym: Harrison and Smyth's syndrome, Subclavian Steal Syndrome, SSS

The subclavian steal phenomenon (SSP) occurs when there is stenosis or occlusion of the subclavian artery proximal to the origin of the vertebral artery. This may cause flow reversal in the ipsilateral vertebral artery as blood is 'stolen' from the circular vertebro-basilar system, to supply the distal territory of the occluded or stenosed artery. Retrograde flow in the vertebral artery, associated with a subclavian or innominate (brachiocephalic) artery stenosis, can be an incidental finding during doppler US examination of the cerebral supply.

The term 'subclavian steal syndrome' should strictly be applied only to cases where this aberrant blood flow causes neurological symptoms. These are related to reduced cerebral perfusion when the arm ipsilateral to the subclavian stenosis is exercised.

Anatomy and blood flow in SSP

See image of normal anatomy of aortic arch and its branches.1
Subclavian steal phenomenon affects the left side much more commonly than the right, with relative incidence about 3-4:1.2,3 This is due to anatomical differences that means the left subclavian artery is much more commonly affected by atherosclerosis.
See diagrams of aberrant flow patterns in left and right subclavian steal phenomena.1

Note, if the left vertebral artery arises directly from the aortic arch (as it does in 2% of population), stenosis of the proximal left subclavian artery cannot cause the syndrome because there is no communication between the vertebral and subclavian arteries.

Epidemiology

Prevalence

This is a relatively uncommon disorder: subclavian stenosis affects about 2% of the general population and 7% of the clinical population.4 SSP is seen as incidental finding on sonography of vertebral system in about 2–5% of those examined. Of these, only about 5% of these patients suffer symptoms.2

Risk factors4

As the majority of cases are due to atherosclerosis, risk factors for SSP are as for cardiovascular disease (CVD) in general:

In Asia, a significant proportion of SSP (36%) is caused by Takayasu's arteritis. These tend to present at a much earlier age (< 30 years) and have a female predominance.2 Takayasu's arteritis is a very rare disease in Europe.5

Presentation

Symptoms

Seek a history of a provoking event that is clearly linked to symptoms. These may be reproducible. Symptoms are usually related to vertebrobasilar and posterior cerebral circulation ischaemia.

On exercising the upper limb on the affected side, the patient may experience any of the following:

  • Vertigo
  • Visual loss ranging from unilateral visual field loss (amaurosis fugax) to bilateral total blindness
  • Transient periods of ataxia, diplopia, dysphagia and dysarthria
  • Tingling or numbness of the face, sensory hemianaesthesia affecting body or transient hemiparesis
  • Hand ischaemia causing arm claudication and rest pain (not a usual feature, consider atheroembolism as a cause)
  • Drop attacks (syncope) - fall to the ground without warning, ± temporary loss of consciousness with immediate recovery6
  • Pulsatile tinnitus7

Neck movements may also cause symptoms.

Signs

  • BP is decreased (>20 mm Hg) in the affected arm distal to the steno-occlusive disease. Check radial and ulnar pulses and elevate the arm, where they may be felt to diminish. It is unusual for a case of genuine subclavian steal syndrome to have no difference in blood pressure between the two arms.
  • Look for a subclavian bruit.
Differential diagnosis
Investigations
  • Doppler ultrasound or angiography shows retrograde flow down vertebral artery (often an asymptomatic, incidental finding).
  • CT or MRI to exclude intracerebral lesion and show any infarcts.
Management

Angioplasty or surgery can both be used to bypass the stenosis of the subclavian artery. Both carry a risk of stroke and death - angioplasty 3.6%, surgery 0.4-2.4% (death only) and recurrent attacks follow surgical treatment in 10-24% of patients. Where symptoms are not severe, conservative management is usually recommended.2

Prognosis

Symptoms may spontaneously resolve due to the establishment of extracranial collaterals to the subclavian circulation. This makes the decision to treat a symptomatic patient not clear cut: treatment is usually reserved for patients with debilitating vertebrobasilar transient ischaemic attacks.
More generally, subclavian stenosis is significantly associated with increased total and CVD-related death, independent of CVD risk factors and existent CVD at diagnosis.8

Associated steal syndromes
  1. Coronary-subclavian steal syndrome: usually iatrogenic and follows coronary artery bypass grafting utilising the internal mammary artery.9 Subclavian stenosis causes 'stealing' of coronary blood flow via the arterial anastomosis, causing angina.
  2. Spinal artery steal syndrome: this very rare condition occurs due to vertebral artery flow reversal, to supply blood to the spinal cord, caused by proximal vertebral artery occlusion.
Historical
  • Whilst Luigi Contorni is credited with describing the collateral circulation in 1960, subsequently described with the neurological symptoms associated with arm exercise by Reivich in 1961, and later termed "subclavian steal syndrome", Contorni himself proposed that the syndrome be named Harrison and Smyth's syndrome in honour of the two 19th century surgeons who originally identified the importance of the collateral circulation and its clinical effects.10
  • Robert Harrison (1796-1858) a student of Colles, was Professor of Anatomy and Physiology in Dublin, and succeeded Colles as Professor of Anatomy in 1837. His textbook for students "Surgical Anatomy of Arteries" in 1829, clearly describes the collateral circulation after occlusion of the first part of the subclavian artery.
  • Andrew Smyth (1833-1916) was an Irishman who attended medical school in Louisiana and worked in New Orleans, whose case description in the "New Orleans Record" of 1866 correctly identified the effects of inverted vertebral flow on the brain.

Document references
  1. Horrow MM, Stassi J; Sonography of the vertebral arteries: a window to disease of the proximal great vessels. AJR Am J Roentgenol. 2001 Jul;177(1):53-9.; Good images/diagrams of aberrant flow in neck vessels.
  2. Brophy D; Subclavian Steal Syndrome. eMedicine, February 2007.
  3. McIntyre K; Subclavian Steal Syndrome. eMedicine, August 2006.
  4. Shadman R, Criqui MH, Bundens WP, et al; Subclavian artery stenosis: prevalence, risk factors, and association with cardiovascular diseases. J Am Coll Cardiol. 2004 Aug 4;44(3):618-23. [abstract]
  5. Ringleb PA, Strittmatter EI, Loewer M, et al; Cerebrovascular manifestations of Takayasu arteritis in Europe. Rheumatology (Oxford). 2005 Aug;44(8):1012-5. Epub 2005 Apr 19. [abstract]
  6. Brignole M, Alboni P, Benditt D, et al; Guidelines on management (diagnosis and treatment) of syncope. Eur Heart J. 2001 Aug;22(15):1256-306.
  7. Lehmann MF, Mounayer C, Benndorf G, et al; Pulsatile tinnitus: a symptom of chronic subclavian artery occlusion. AJNR Am J Neuroradiol. 2005 Sep;26(8):1960-3. [abstract]
  8. Aboyans V, Criqui MH, McDermott MM, et al; The vital prognosis of subclavian stenosis. J Am Coll Cardiol. 2007 Apr 10;49(14):1540-5. Epub 2007 Mar 26. [abstract]
  9. Costa SM, Fitzsimmons PJ, Terry E, et al; Coronary-subclavian steal: case series and review of diagnostic and therapeutic strategies: three case reports. Angiology. 2007 Apr-May;58(2):242-8. [abstract]
  10. Contorni L; The true story fo the "Subclavian steal syndrome" or "Harrison and Smyth's syndrome". J Cardiovasc Surg (Torino). 1973 Jul-Aug;14(4):408-17.

Internet and further reading Acknowledgements EMIS is grateful to Dr Chloe Borton for writing this article. The final copy has passed scrutiny by the independent Mentor GP reviewing team. ©EMIS 2008.
DocID: 2814
Document Version: 20
DocRef: bgp717
Last Updated: 30 Jan 2008
Review Date: 29 Jan 2010

The authors and editors of this article are employed to create accurate and up to date content reflecting reliable research evidence, guidance and best clinical practice. They are free from any commercial conflicts of interest. Find out more about updating.

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