Severe autonomic failure occurs in about 1 in 1000 people. Such patients are remarkable for the striking and sometimes paradoxical responses they manifest to a variety of physiologic and pharmacologic stimuli. Orthostatic hypotension is often the finding most commonly noted by physicians, but a myriad of additional and less well-understood findings also occur. These include supine hypertension, altered drug sensitivity, hyperresponsiveness of blood pressure to hypo/hyperventilation, sleep apnea and other neurological disturbances are also frequently encountered.
In this article we will review the clinical pathophysiology that underlies autonomic failure, with a particular emphasis on those aspects most relevant to the care of such patients in the perioperative settings. Strategies used by clinicians to diagnose and treat these patients, and the impact of these interventions on the pre-operative, intra-operative, and post-operative care that these patients undergo is a crucial element in the optimized management of care in these patients.