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Dysautonomiaperioperative Implications REVIEW ARTICLE David S. Warner, M.D., Editor Dysautonomia Perioperative Implications Hossam I. Mustafa, M.D., M.S.C.I.,* Joshua P. Fessel, M.D., Ph.D.,† John Barwise, M.D.,‡ John R. Shannon, M.D.,§ Satish R. Raj, M.D., M.S.C.I.,ʈ Andre´ Diedrich, M.D., Ph.D.,# Italo Biaggioni, M.D.,** David Robertson, M.D.†† This article has been selected for the ANESTHESIOLOGY CME Program. Learning objectives and disclosure and ordering information can be found in the CME section at the front of this issue. ABSTRACT physiologic and pharmacologic stimuli. Orthostatic hypo- tension is often the finding most commonly noted by physi- Severe autonomic failure occurs in approximately 1 in 1,000 cians, but a myriad of additional and less understood find- people. Such patients are remarkable for the striking and ings also occur. These findings include supine hypertension, sometimes paradoxic responses they manifest to a variety of altered drug sensitivity, hyperresponsiveness of blood pres- sure to hypo/hyperventilation, sleep apnea, and other neuro- * Research Fellow, Autonomic Dysfunction Center, Division of Clin- logic disturbances. ical Pharmacology, Departments of Medicine and Pharmacology, Au- In this article the authors will review the clinical patho- tonomic Rare Disease Clinical Research Consortium, Vanderbilt Uni- physiology that underlies autonomic failure, with a particu- versity Medical Center, Nashville, Tennessee. † Assistant Professor, Autonomic Dysfunction Center, Divisions of Pulmonary and Critical lar emphasis on those aspects most relevant to the care of Care Medicine, Departments of Medicine and Pharmacology, Auto- such patients in the perioperative setting. Strategies used by nomic Rare Disease Clinical Research Consortium, Vanderbilt Univer- clinicians in diagnosis and treatment of these patients, and sity Medical Center. ‡ Assistant Professor, Department of Anesthesiol- ogy, Autonomic Rare Disease Clinical Research Consortium, Vanderbilt the effect of these interventions on the preoperative, intraop- University Medical Center. § Assistant Professor, Autonomic Dysfunction erative, and postoperative care that these patients undergo is Center, Division of Clinical Pharmacology, Departments of Medicine and a crucial element in the optimized management of care in Anesthesiology, Autonomic Rare Disease Clinical Consortium, Vanderbilt University Medical Center. ʈ Assistant Professor, Autonomic Dysfunction these patients. Center, Division of Clinical Pharmacology, Department of Medicine and Pharmacology, Autonomic Rare Disease Clinical Research Consortium, Vanderbilt University Medical Center. # Associate Professor, Autonomic EVERE autonomic failure occurs in approximately 1 in 1,2 Dysfunction Center, Division of Clinical Pharmacology, Department of S 1,000 people. Such patients are remarkable for the Biomedical Engineering, Autonomic Rare Disease Clinical Research Con- striking and sometimes paradoxic responses they manifest to sortium, Vanderbilt University Medical Center. ** Professor, Autonomic Dysfunction Center, Division of Clinical Pharmacology, Departments of a variety of physiologic and pharmacologic stimuli. Auto- Medicine and Pharmacology, Autonomic Rare Disease Clinical Research nomic failure may be secondary to other diseases, such as Consortium, Vanderbilt University Medical Center. †† Professor, Auto- diabetes mellitus, amyloidosis, or bronchogenic carcinoma, nomic Dysfunction Center, Division of Clinical Pharmacology, Depart- ments of Medicine, Pharmacology, and Neurology, Autonomic Rare Dis- or be due to a primary autonomic disorder such as multiple ease Clinical Research Consortium, Vanderbilt University Medical Center. system atrophy (MSA) such as Shy-Drager syndrome, or Received from the Department of Medicine, Divisions of Clinical pure autonomic failure. Pharmacology/Pulmonary and Critical Care Medicine and the Depart- ment of Anesthesiology, Vanderbilt University Medical Center, Nash- ville, Tennessee. Submitted for publication June 28, 2010. Accepted for Address correspondence to Dr. Robertson: Elton Yates Professor of publication August 4, 2011. Supported by P01 HL056693 (to Dr. Rob- Medicine, Pharmacology and Neurology, and Director, Clinical and ertson), R01 HL071784 (to Dr. Robertson), K23 RR020783 (to Dr. Raj), Translational Research Center, Division of Clinical Pharmacology, De- the Vanderbilt CTSA (UL1 RR024975), and the Autonomic Diseases partments of Medicine, Pharmacology, and Neurology, Vanderbilt Uni- Clinical Research Consortium U54 NS065736 (to Dr. Robertson), all versity Medical Center, 3228 Medical Center North, Nashville, Tennes- from the National Institutes of Health, Bethesda, Maryland. see 37232-2195. [email protected]. This article may be Copyright © 2011, the American Society of Anesthesiologists, Inc. Lippincott accessed for personal use at no charge through the Journal Web site, Williams & Wilkins. Anesthesiology 2012; 116:205–15 www.anesthesiology.org. Anesthesiology, V 116 • No 1 205 January 2012 Downloaded From: http://anesthesiology.pubs.asahq.org/pdfaccess.ashx?url=/data/Journals/JASA/931114/ on 10/05/2016 Autonomic Failure and Anesthesia The authors will review the pathophysiology that under- Table 1. Commonplace Stimuli that Can Cause Blood lies autonomic failure, with a particular emphasis on those Pressure Changes in Autonomic Failure aspects most relevant to the care of such patients in the peri- Decreases Blood Pressure Increases Blood Pressure operative and intraoperative settings. The authors will also discuss strategies that can be used to manage the symptoms of Standing Lying supine autonomic failure. Food Water ingestion Proper functioning of the autonomic nervous system re- Hyperventilation Hypoventilation Straining (Valsalva) Abdominal binding quires that both afferent and efferent limbs are intact. Affer- Fever and heat Coffee ent neurons detect changes in blood pressure, temperature, Dehydration Water immersion and the myriad other vital processes controlled by the auto- Exercise Head-down tilt nomic nervous system, and communicate these changes cen- trally, whereas the efferent neurons engage effector systems sis and worsens orthostatic hypotension, often complicating to perturb or restore homeostasis. Dysfunction of the affer- management. Supine hypertension is characterized by an in- ent limb is typically associated with labile hypertension, as crease in peripheral vascular resistance. This is due to the seen in baroreflex failure, particularly during the postopera- residual sympathetic tone in patients with MSA, but the tive period after endarterectomy or other neck surgeries af- cause is not clear in patients with pure autonomic failure who fecting the carotid sinus nerve.3 Abnormalities of central au- have similarly increased vascular resistance despite very low tonomic pathways such as in patients with MSA, of efferent 4,5 levels of plasma norepinephrine and renin activity. Due to effector systems as in patients with pure autonomic failure, absent or decreased baseline efferent neural traffic, patients ␤ hydroxylase, or any patients with deficiency of dopamine with autonomic failure may also manifest denervation hyper- combination thereof all can lead to clinical autonomic failure sensitivity to norepinephrine. Denervation hypersensitivity and disabling orthostatic hypotension. results in hyperresponsiveness of vascular smooth muscles to Some of the most severely affected individuals have pure the chemical mediator that normally activates it (in this case, autonomic failure or multiple system atrophy. Such patients norepinephrine), due in part to up-regulation of adrenergic often have both extremely low blood pressures while upright receptors secondary to the low levels of plasma norepineph- and extremely high blood pressures while supine. Patients 6,7 rine in autonomic failure. with MSA are perhaps the most vulnerable of all such pa- tients to the complex and interacting responses to drugs and Basic Evaluation of Patients with Autonomic perturbations that occur during anesthesia. Dysfunction Preoperative Evaluation of Autonomic Basic evaluation of patients includes obtaining a detailed Dysfunction clinical history and examination, and measurement of blood pressure and heart rate while supine and again after 1 and 3 Common Features Associated with Autonomic min of standing. Blood pressure measured while seated can Dysfunction sometimes be useful for those patients who cannot stand for Orthostatic hypotension is the core feature of autonomic even 1 min. More detailed autonomic function testing is failure. Orthostatic hypotension is defined as a decrease in usually performed in specialized centers, using multiples blood pressure of 20/10 mmHg on standing for 3 min. This methods such as the following procedures: orthostatic and definition is meant to capture initial or mild cases. Symp- tilt-table testing; blood pressure and heart rate are measured tomatic orthostatic hypotension is more commonly encoun- twice in the supine position. The patient then is instructed to tered in subjects with decreases in blood pressure that may stand for 5 min. Blood pressure and heart rate are again range from this figure to the severest cases where the decrease determined at 1, 3, and 5 min in the upright position. In in blood pressure may be 100/50 mmHg. The most common most instances of autonomic failure, there may be little or no symptoms of orthostatic hypotension are lightheadedness, increase in heart rate even though the blood pressure may de- dimming/tunneling
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