Recurrent Subclavian Steal Syndrome: A Novel Case of Vasculopathy

Subclavian steal syndrome (SSS) refers to the phenomenon of retrograde flow in an ipsilateral branch of the subclavian artery due to hemodynamically significant stenosis or occlusion of the ipsilateral proximal subclavian artery. While SSS is usually asymptomatic, it can manifest as vertebrobasilar insufficiency (VBI), ischemia of the affected extremity, or cardiac angina when an internal mammary artery (IMA) is used as a bypass graft. The underlying etiology is most often atherosclerosis but can include Takayasu arteritis, thoracic outlet syndrome, cervical rib, and stenosis secondary to surgical repair of aortic coarctation or tetralogy of Fallot. There are several case reports describing unique presentations of SSS as well as limited reports of double SSS, where the brachiocephalic steno-occlusive disease causes flow reversal in both the ipsilateral vertebral and carotid arteries. We report herein the first documented case, to our knowledge, of a patient with SSS previously treated with left subclavian artery stenting and left common carotid-subclavian bypass who developed recurrent SSS in conjunction with orthostatic cerebral hypoperfusion syndrome (OCHOS) secondary to severe vasculopathy. She presented with recurrent, paroxysmal vertigo and near-syncope associated with left upper extremity paresthesias that would only abate with sitting in the context of left subclavian artery stent restenosis and occlusion of her left common carotid-subclavian bypass graft. Interestingly, her initial presentation entailed retrograde flow from the left vertebral artery to the left subclavian artery, classic for SSS, but recurrence of her SSS involved retrograde flow from the left common carotid artery to the left subclavian artery, a phenomenon which has also not been described in the literature to our knowledge. As her symptoms of VBI appeared to be triggered by standing and not left arm movement, they were considered to be primarily secondary to OCHOS. Consequently, her primary treatment was to increase salt and fluid intake and thus increase intravascular volume for improved cerebral perfusion as she was not deemed to be a suitable candidate for regrafting of the left subclavian artery.