The Many Faces of “Pulseless Disease”

Abstract

“Pulseless disease” is a term which was originated to describe an obliterative arteritis of the aortic arch occurring in young women. The expression has subsequently been corrupted by popular usage to apply to almost any condition which results in the diminution or absence of pulses in the neck or upper extremity. The resulting confusion in the medical literature has been compounded by the substitution of a variety of alternative names which are not truly synonomous with each other. No one name and no one list of general descriptive features can adequately encompass all patients who exhibit pulse deficits in the neck or arms. In order to understand these cases, it is necessary to consider them according to their underlying etiology and the extent of arterial obstruction. In the United States, obstruction of flow through the aortic arch vessels is most commonly due to atherosclerosis. Syphilitic arteritis and “non-specific” arteritis also produce occlusions of these vessels, but their incidence is comparatively low. The characteristic stenosing inflammatory arteritis seen in young women of Asia or northern Europe (Takayasu's disease) is a distinct rarity in this country. The historic aspects, symptoms, physical signs, prognosis and current treatment of this group of diseases have been briefly summarized. Aortic arch angiography is the most accurate means of determining the extent of arterial obstruction. Direct arterial reconstruction is the treatment of choice in patients who have not yet suffered irreversible neurologic deficits and whose general medical status does not contraindicate thoracotomy.