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2020-Baroreflex-Dysfunction.Pdf The new england journal of medicine Review Article Dan L. Longo, M.D., Editor Baroreflex Dysfunction Horacio Kaufmann, M.D., Lucy Norcliffe‑Kaufmann, Ph.D., and Jose‑Alberto Palma, M.D., Ph.D.​​ he autonomic nervous system innervates all body organs, in- From the Department of Neurology, Dys‑ cluding the cardiovascular system. Smooth muscle in arteries, arterioles, autonomia Center, New York University School of Medicine, New York. Address and veins and pericytes in capillaries receive autonomic innervation, which reprint requests to Dr. Kaufmann at the T Dysautonomia Center, NYU Langone modulates vascular smooth-muscle tone and vessel diameter. Afferent sensory neurons with receptors monitoring local changes in the chemical and mechanical Health, 530 First Ave., Suite 9Q, New York, NY 10016, or at horacio . kaufmann@ environment provide the information that allows the autonomic nervous system to nyulangone . org. regulate blood flow within every organ and redirect cardiac output to vascular N Engl J Med 2020;382:163-78. beds as needed. The autonomic nervous system provides moment-to-moment control DOI: 10.1056/NEJMra1509723 1 of blood pressure and heart rate through baroreflexes. These negative-feedback Copyright © 2020 Massachusetts Medical Society. neural loops regulate specific groups of both sympathetic neurons sending nerve impulses to the vasculature, the heart, and the kidney and parasympathetic neurons sending nerve impulses to the sinus node of the heart. We describe the main features of baroreflexes and the clinical phenotypes of baroreflex impairment. Baroreflexes Baroreflexes enable the circulatory system to adapt to varying conditions in daily life while maintaining blood pressure, heart rate, and blood volume within a narrow physiologic range. Baroreceptors embedded in the walls of major arteries and veins and the heart elicit distinct reflexes.2 They continuously signal to the nucleus of the solitary tract, located in the brain stem, through the vagus and glossopharyngeal nerves and are activated by stretch when blood pressure, blood volume, or both rise (Fig. 1). To counter the rise, baroreceptors evoke reflex inhibition of efferent sympathetic signals to splanchnic, skeletal-muscle, and renal blood vessels, causing vasodilatation. A concomitant increase in parasympathetic-nerve traffic to the sino- atrial node slows the heart rate. Conversely, when a change to a standing position is made, baroreceptors are unloaded, allowing vasoconstriction and tachycardia to buffer the fall in blood pressure that would otherwise occur. Not infrequently, there is initial orthostatic hypotension caused by a transient mismatch between cardiac output and peripheral vascular resistance, which quickly rebounds. Arterial baroreceptors in the carotid sinuses and aortic arch sense pressure changes, and cardiopulmonary baroreceptors in thoracic veins and the heart sense changes in blood volume. Both arterial and cardiopulmonary baroreceptors inhibit efferent sympathetic neurons, leading to vasodilatation, but only arterial barore- ceptors influence the heart rate. Arterial baroreceptors preferentially target the splanchnic circulation, and cardiopulmonary baroreceptors inhibit sympathetic renal outflow, reducing renin release and proximal tubular sodium reabsorption. Baroreceptor activation also suppresses vasopressin release and sodium appetite, increasing urine output (Fig. 1). Mechanosensing by arterial baroreceptors is medi- ated by the mechanically activated excitatory ion channels PIEZO1 and PIEZO2.3 n engl j med 382;2 nejm.org January 9, 2020 163 The New England Journal of Medicine Downloaded from nejm.org by JESUS RUEDA on February 18, 2020. For personal use only. No other uses without permission. Copyright © 2020 Massachusetts Medical Society. All rights reserved. The new england journal of medicine AFFERENTS CENTRAL EFFERENTS High-pressure Paraventricular baroreceptors nucleus Supraoptic (arterial) nucleus Carotid AVP Aortic Low-pressure baroreceptors (cardiopulmonary) Rostral ventrolateral Glossopharyngeal medulla Nucleus NTS ambiguus Pulmonary Parasympathetic Atrial neurons Vagus SAN Ventricular CVLM ACh Sympathetic NE ganglia ACh IML NE Dorsal-root Renin ganglia NE NE Preganglionic Postganglionic Sympathetic neurons Figure 1. Neuroanatomy of Baroreflexes. Afferent baroreflex mechanosensing neurons with axonal receptors in thoracic arteries (aortic arch and carotid sinuses) and the heart have their cell bodies in the nodose and petrosal ganglia (yellow ovals) of the glossopharyngeal and vagal nerves (nerves IX and X, respectively) and synapse with neurons in the brain‑stem medulla oblongata at the nucleus of the solitary tract (NTS). From the NTS, there are three baroreflex pathways. The first is an inhibitory pathway that restrains sympathetic outflow to the vasculature. Through interneurons in the caudal ventrolateral medulla (CVLM), neurons in the NTS inhibit sympathetic (premotor) pacemaker neurons in the rostral ventrolateral medulla. The neurons in the rostral ventrolateral medulla are the source of sympathetic activity to the vasculature and are organized in groups that preferentially or exclusively control the movement of sympathetic efferent nerves to specific vascular beds. Axons of barosensitive neurons in the rostral ventrolateral medulla descend through the spinal cord and activate the two‑neuron (preganglionic and postganglionic) sympathetic efferent pathway to skeletal muscle and mesenteric and renal vessels. The second path‑ way activates vagal efferents to slow the heart rate through a direct projection to preganglionic parasympathetic neurons in the nucleus ambiguus of the medulla, which activate postganglionic parasympathetic neurons to the sinoatrial node. The third pathway connects the NTS with the supraoptic nucleus) and the paraventricular nucleus in the hypothalamus, controlling arginine vasopressin (AVP) release from the pituitary. Also shown are renal afferents and muscle ergoreceptors (thinly myelinated group III and IV afferents), which reach the cord through the dorsal‑root ganglia and have important modulatory effects on the baroreflex. ACh denotes acetylcholine, IML inter‑ mediolateral cell column, NE norepinephrine, and SAN sinoatrial node. 164 n engl j med 382;2 nejm.org January 9, 2020 The New England Journal of Medicine Downloaded from nejm.org by JESUS RUEDA on February 18, 2020. For personal use only. No other uses without permission. Copyright © 2020 Massachusetts Medical Society. All rights reserved. Baroreflex Dysfunction Baroreflexes during the Cardiac Cycle, or high thoracic spinal cord lesions, autonomic Respiratory Entrainment, and Other Inputs dysreflexia results in a similar phenomenon when At rest, afferent baroreceptor discharge on the stimuli from the viscera or skin trigger abnor- nucleus of the solitary tract maintains a tonic mal spinal sympathetic activation.8,9 So-called level of peripheral sympathetic inhibition and functional autonomic disorders are encountered cardiovagal activation. In synchronicity with the most frequently in the clinic and are not associ- pulse wave, afferent baroreceptor discharge in- ated with any detectable nerve disease. The esti- creases with each systole and decreases during mated prevalence in the United States of most of diastole, causing reciprocal changes in sympa- the disorders affecting baroreflexes is shown in thetic and vagal efferent activity. Vagal efferent Table 1. neurons also entrain with respiratory neurons and are inhibited during inspiration, giving rise Efferent Baroreflex Failure to respiratory sinus arrhythmia. When the baro- reflex pathways are damaged, these physiologic Diseases affecting baroreflex sympathetic effer- rhythms are lost or blunted. ent neurons impair the release of norepinephrine The nucleus of the solitary tract also receives at the neurovascular junction. Insufficient vaso- and integrates information from other sources, constriction on standing or exertion leads to ortho- including peripheral and central chemorecep- static hypotension and symptoms of organ hypo- tors, renal mechanoreceptors and chemoreceptors perfusion, including lightheadedness or dizziness, through renal afferent nerves, muscle ergorecep- visual blurring, and syncope. Dyspnea, subtle tors (muscle afferents that are stimulated by mus- cognitive slowing, and fatigue are common and cle work) (Fig. 1), and respiratory neurons, as well disappear in the supine position. Clinically, this as cortical and hypothalamic neurons. Increased disorder is defined by a sustained fall in blood input from chemoreceptors heightens sympathetic pressure of at least 20/10 mm Hg within 3 min- outflow in congestive heart failure, pulmonary utes after assumption of an upright posture,18 hypertension, and chronic obstructive pulmonary but in some cases, the fall in blood pressure is disease,4-6 and altered renal afferent activity may delayed, occurring after prolonged standing.19 underlie sympathetic-nerve excitation in arterial Supine hypertension (blood pressure, >140/90 hypertension. mm Hg) develops in 50% of patients with effer- ent baroreflex failure, probably as a result of the Baroreflex Impairment activation of residual sympathetic fibers and de- Diseases affecting baroreflex neurons cause un- nervation supersensitivity.20 Loss of the normal stable blood pressure with acute symptoms of nocturnal profile of blood-pressure dipping im- hypoperfusion or hyperperfusion. Lesions of af- pairs extracellular fluid-volume regulation. It is ferent, central,
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