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Libby: Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 8th ed.
Copyright © 2007 Saunders, An Imprint of Elsevier

CHAPTER 37 – Hypotension and Syncope
Hugh Calkins Douglas P. Zipes
CHAPTER OUTLINE

Definition, 975 Classification, 975 Vascular Causes of Syncope, 975 Cardiac Causes of Syncope, 978 Neurological Causes of Apparent Syncope, 978 Metabolic Causes of Apparent Syncope, 978 Relationship Between Prognosis and Cause of Syncope, 978 Evaluation, 978 History and Physical Examination, 978 Diagnostic Tests, 979 Approach to the Evaluation of Patients with Syncope, 981 Management, 982 Neurally Mediated Syncope, 983 References, 983

DEFINITION
Syncope is a sudden transient loss of consciousness and postural tone with spontaneous recovery. Loss of consciousness results from a reduction of blood flow to the reticular activating system located in the brain stem and does not require electrical or chemical therapy for reversal. The metabolism of the brain, in contrast to that of many other organs, is exquisitely dependent on perfusion. Consequently, cessation of cerebral blood flow leads to loss of consciousness within approximately 10 seconds. Restoration of appropriate behavior and orientation after a syncopal episode is usually immediate. Retrograde amnesia is uncommon. Syncope is an important clinical problem because it is common, costly, often disabling, may cause injury, and may be the only warning sign before sudden cardiac death (see Chap. 36) . [1] Patients with syncope account for 1 percent of hospital admissions and 3 percent of emergency department visits. Elderly persons have a 6 percent annual incidence of syncope (see Chap. 75) . Surveys of young adults have revealed that up to 50 percent report a prior episode of loss of consciousness; most of these episodes are isolated events that never come to medical attention. The Framingham Study, in which biennial examinations were performed on 7814 individuals, reported the incidence of a first report of syncope to be 6.2/1000 person-years follow-up. [2] Patients who experience syncope also report a markedly reduced quality of life. The prognosis of patients with syncope varies greatly with the diagnosis. In the Framingham Study, for example, participants with syncope, including those with syncope of unknown origin, had increased mortality compared with participants without syncope. The highest mortality was observed among those with a cardiac cause of syncope. In

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contrast, the subgroup of participants with neurally mediated syncope (including orthostatic hypotension and medication-related syncope) did not experience increased mortality. [2]
Classification

The causes of apparent syncopal episodes can be separated initially into two groups—true syncope, in which the transient loss of consciousness results from cerebral hypoperfusion, and nonsyncopal conditions, with real or apparent loss of consciousness from other causes. [1] Table 37-1 shows the differential diagnosis of syncope and nonsyncopal conditions, which are indicated by an asterisk. Vascular causes of syncope are most common, followed by cardiac causes. Among cardiac causes of syncope, arrhythmias are most common. The importance of considering nonsyncopal conditions when evaluating a patient with apparent loss of consciousness cannot be underestimated. These nonsyncopal conditions include conditions in which consciousness is lost as a result of metabolic disorders, epilepsy, or alcohol, as well as conditions in which consciousness is only apparently lost (i.e., conversion reaction). These psychogenic causes of syncope are now being recognized with increased frequency. TABLE 37-1 -- Causes of Syncope Vascular Anatomical Vascular steal syndromes Orthostatic Autonomic insufficiency Idiopathic Volume depletion Drug- and alcoholinduced Reflex-mediated Carotid sinus hypersensitivity Neurally mediated syncope Glossopharyngeal syncope Situational (cough, sneeze, swallow, micturition, postprandial) Cardiac Anatomical Obstructive cardiac valvular disease Aortic dissection Atrial myxoma Pericardial disease, tamponade Hypertrophic obstructive

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cardiomyopathy Myocardial ischemia, infarction Pulmonary embolus Pulmonary hypertension Arrhythmias Bradyarrhythmias Sinus node dysfunction, bradycardia Atrioventricular block Tachyarrhythmias Supraventricular arrhythmias Ventricular arrhythmias (including long-QT and Brugada syndromes) Implanted pacemaker or implantable cardioverter-defibrillator malfunction Neurological and cerebrovascular [*] Arnold-Chiari malformation Migraine Seizure (partial complex, temporal lobe) Vertebrobasilar insufficiency/transient ischemic attack Metabolic [*] Drugs, alcohol Hyperventilation (hypocapnia) Hypoglycemia Hypoxemia Psychogenic syncope [*] Anxiety, panic disorder Somatization disorders Syncope of unknown origin
*

Disorders resembling syncope.

Although knowledge of the common conditions that can cause syncope is essential and allows the clinician to arrive at a probable cause of syncope in most patients, it is equally important to be aware of several of the less common but potentially lethal causes of syncope, such as the long-QT

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syndrome, arrhythmogenic right ventricular cardiomyopathy, Brugada syndrome, idiopathic ventricular fibrillation, catecholaminergic polymorphic ventricular tachycardia, short-QT syndrome (see Chap. 9) , hypertrophic cardiomyopathy (see Chap. 65) , and pulmonary emboli (see Chap. 72) . [3] [4] [5] [6] [7] [8] [9] [10] It is also important to recognize that distribution of the causes of syncope varies with age. In young individuals, neurally mediated syncope is the most common, but is an unusual type of syncope in elderly persons. [11] Common causes of syncope in elderly persons include orthostatic hypotension, postprandial hypotension, medication, aortic stenosis (see Chap. 62) , carotid sinus hypersensitivity, and bradyarrhythmias (e.g., sick sinus syndrome, heart block; see Chap. 35 ).
Vascular Causes of Syncope

Vascular causes of syncope, particularly reflex-mediated syncope and orthostatic hypotension, are the most common causes, accounting for at least one third of all syncopal episodes. [1] [2] [11] In contrast, vascular steal syndromes are exceedingly uncommon causes of syncope.
Orthostatic Hypotension

When a person stands, 500 to 800 ml of blood is displaced to the abdomen and lower extremities, resulting in an abrupt drop in venous return to the heart. This drop leads to a decrease in cardiac output and stimulation of aortic, carotid, and cardiopulmonary baroreceptors, which trigger a reflex increase in sympathetic outflow. As a result, heart rate, cardiac contractility, and vascular resistance increase to maintain a stable systemic blood pressure on standing. Orthostatic hypotension, which is defined as a 20-mmHg drop in systolic blood pressure or a 10-mmHg drop in diastolic blood pressure within 3 minutes of standing, results from a defect in any portion of this blood pressure control system. Orthostatic hypotension can be asymptomatic or associated with symptoms such as lightheadedness, dizziness, blurred vision, weakness, palpitations, tremulousness, and syncope. These symptoms are often worse immediately on arising in the morning or after meals or exercise. Syncope that occurs after meals, particularly in elderly people, can result from a redistribution of blood to the gut. A decline in systolic blood pressure of about 20 mmHg approximately 1 hour after eating has been reported in up to one third of elderly nursing home residents. Although usually asymptomatic, it can result in lightheadedness or syncope. Drugs that cause volume depletion or result in vasodilation are the most common cause of orthostatic hypotension ( Table 37-2 ). Elderly patients are particularly susceptible to the hypotensive effects of drugs because of reduced baroreceptor sensitivity, decreased cerebral blood flow, renal sodium wasting, and an impaired thirst mechanism that develops with aging. Orthostatic hypotension can also result from neurogenic causes, which can be subclassified into primary and secondary autonomic failure. [12] Primary causes are generally idiopathic, whereas secondary causes are associated with a known biochemical or structural anomaly or are seen as part of a particular disease or syndrome. There are three types of primary autonomic failure (see Chap. 89) . Pure autonomic failure (Bradbury-Eggleston syndrome) is an idiopathic sporadic disorder characterized by orthostatic hypotension, usually in conjunction with evidence of more widespread autonomic failure, such as disturbances in bowel, bladder, thermoregulatory, and sexual function. Patients with pure autonomic failure have reduced supine plasma norepinephrine levels. Multiple system atrophy (Shy-Drager syndrome) is a sporadic, progressive, adult-onset disorder characterized by autonomic dysfunction, parkinsonism, and ataxia in any combination. The third type of primary autonomic failure is Parkinson disease with autonomic failure. A small subset of patients with Parkinson disease may also experience autonomic failure, including orthostatic hypotension. In addition to these forms of chronic autonomic failure is a rare acute panautonomic neuropathy. This neuropathy generally occurs in young people and results in widespread severe sympathetic and parasympathetic failure, with orthostatic hypotension, loss of

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sweating, disruption of bladder and bowel function, fixed heart rate, and fixed dilated pupils. TABLE 37-2 -- Causes of Orthostatic Hypotension Drugs Diuretics Alpha-adrenergic blocking drugs—terazosin (Hytrin), labetalol Adrenergic neuron blocking drugs—guanethidine Angiotensin-converting enzyme inhibitors Antidepressants Monoamine oxidase inhibitors Alcohol Diuretics Ganglion-blocking drugs—hexamethonium, mecamylamine Tranquilizers—phenothiazines, barbiturates Vasodilators—prazosin, hydralazine, calcium channel blockers Centrally acting hypotensive drugs—methyldopa, clonidine Primary Disorders of Autonomic Failures Pure autonomic failure (Bradbury-Eggleston syndrome) Multisystem atrophy (Shy-Drager syndrome) Parkinson disease with autonomic failure Secondary Neurogenic Causes Aging Autoimmune disease Guillain-Barré syndrome, mixed connective tissue disease, rheumatoid arthritis Eaton-Lambert syndrome, systemic lupus erythematosus Carcinomatosis autonomic neuropathy Central brain lesions Multiple sclerosis, Wernicke encephalopathy Vascular lesions or tumors involving the hypothalamus and midbrain Dopamine β-hydroxylase deficiency Familial hyperbradykininism General medical disorders—diabetes, amyloidosis, alcoholism, renal failure

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Hereditary sensory neuropathies, dominant or recessive Infections of the nervous system—human immunodeficiency virus infection, Chagas' disease, botulism, syphilis Metabolic disease—vitamin B12 deficiency, porphyria, Fabry disease, Tangier disease Spinal cord lesions

Adapted from Bannister SR (ed): Autonomic Failure, 2nd ed. Oxford, Oxford University Press, 1988, p 8.
Postural orthostatic tachycardia syndrome (POTS) is a milder form of chronic autonomic failure and orthostatic intolerance characterized by the presence of symptoms of orthostatic intolerance, an increase of 28 beats/min or more in heart rate, and absence of a significant change in blood pressure within 5 minutes of standing or upright tilt. [13] POTS appears to result from a failure of the peripheral vasculature to vasoconstrict appropriately under orthostatic stress. POTS can also be associated with syncope related to neurally mediated hypotension (see later).
Reflex-mediated Syncope

There are many reflex-mediated syncopal syndromes ( Table 37-3 ). In each case, the reflex is composed of a trigger (the afferent limb) and a response (the efferent limb). This group of reflexmediated syncopal syndromes has in common the response limb of the reflex, which consists of increased vagal tone and a withdrawal of peripheral sympathetic tone and leads to bradycardia, vasodilation and, ultimately, hypotension, presyncope, or syncope. What distinguishes these causes of syncope are the specific triggers. For example, micturition syncope results from activation of mechanoreceptors in the bladder, defecation syncope results from neural inputs from gut wall tension receptors, and swallowing syncope results from afferent neural impulses arising from the upper gastrointestinal tract. The two most common types of reflex-mediated syncope, carotid sinus hypersensitivity and neurally mediated hypotension, are discussed later. TABLE 37-3 -- Differentiating Syncope Caused by Neurally Mediated Hypotension, Arrhythmias, and Seizures Neurally Mediated Parameter Arrhythmia Seizure Hypotension Demographics, clinical setting Female > male gender Younger age (<55 yr) More episodes (more than two) Male > female gender Older age (>54 yr) Fewer episodes (less than three) Younger age (<45 yr) Any setting

Standing, warm room, During exertion, or supine emotional upset Family history of sudden death Premonitory symptoms Longer duration (>5 sec) Shorter duration (<6 sec) Sudden onset or brief aura (déjà vu, olfactory, gustatory, visual)

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Palpitations Blurred vision Nausea Warmth Diaphoresis Lightheadedness Observations during the event Pallor Diaphoretic Dilated pupils Slow pulse, low blood pressure Incontinence—may occur Brief clonic movements—may occur

Palpitations less common

Blue, not pale Incontinence can occur. Brief clonic movements can occur.

Blue face, no pallor Frothing at the mouth Prolonged syncope (duration > 5 min) Tongue biting Horizontal eye deviation Elevated pulse and blood pressure Incontinence more likely
[*]

Tonic-clonic movements if grand mal Residual symptoms Residual symptoms common Prolonged fatigue common (>90%) Oriented Oriented Residual symptoms uncommon (unless prolonged unconsciousness) Residual symptoms common Aching muscles Disoriented Fatigue Headache Slow recovery
*

May be observed with any of these causes of syncope but more common with seizures.

NEURALLY MEDIATED HYPOTENSION OR SYNCOPE.

The term neurally mediated hypotension or syncope (also known as neurocardiogenic, vasodepressor, and vasovagal syncope and as “fainting”) has been used to describe a common abnormality of blood pressure regulation characterized by the abrupt onset of hypotension with or without bradycardia. Triggers associated with the development of neurally mediated syncope are those that reduce ventricular filling or increase catecholamine secretion. [1] [14] They include the sight of blood, pain, prolonged standing, a warm environment or hot shower, and stressful situations. In these types of situations, patients with this condition experience severe lightheadedness, syncope, or both. It has been postulated that these clinical phenomena result

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from a paradoxical reflex that is initiated when ventricular preload is reduced by venous pooling. This reduction leads to a reduction in cardiac output and blood pressure, which is sensed by arterial baroreceptors. The resultant increased catecholamine levels, combined with reduced venous filling, lead to a vigorously contracting volume-depleted ventricle. The heart itself is involved in this reflex by virtue of the presence of mechanoreceptors, or C-fibers, consisting of nonmyelinated fibers found in the atria, ventricles, and pulmonary artery. It has been proposed that vigorous contraction of a volume-depleted ventricle leads to activation of these receptors in susceptible individuals. These afferent C-fibers project centrally to the dorsal vagal nucleus of the medulla, leading to a “paradoxical” withdrawal of peripheral sympathetic tone and an increase in vagal tone, which, in turn, causes vasodilation and bradycardia. The ultimate clinical consequence is syncope or presyncope. Not all neurally mediated syncope results from the activation of mechanoreceptors. In humans, it is well known that the sight of blood or extreme emotion can trigger syncope. These observations suggest that higher neural centers can also participate in the pathophysiology of vasovagal syncope. In addition, central mechanisms can contribute to the production of neurally mediated syncope.
CAROTID SINUS HYPERSENSITIVITY.

Syncope caused by carotid sinus hypersensitivity results from stimulation of carotid sinus baroreceptors, located in the internal carotid artery above the bifurcation of the common carotid artery. Carotid sinus hypersensitivity is diagnosed by applying gentle pressure over the carotid pulsation just below the angle of the jaw, where the carotid bifurcation is located. Pressure should be applied for 5 to 10 seconds. It is important to perform carotid sinus massage in both the supine and upright positions. The main complications associated with performing carotid sinus massage are neurological. Because of this, carotid sinus massage should be avoided in patients with prior transient ischemic attacks, strokes within the past 3 months, and carotid bruits. A normal response to carotid sinus massage is a transient decrease in the sinus rate, slowing of atrioventricular (AV) conduction, or both. Carotid sinus hypersensitivity is defined as a sinus pause longer than 3 seconds in duration and a fall in systolic blood pressure of 50 mmHg or more. The response to carotid sinus massage can be classified as cardioinhibitory (asystole), vasodepressive (fall in systolic blood pressure), or mixed. Carotid sinus hypersensitivity is detected in approximately one third of elderly patients who present with syncope or after falls. [1] [15] It is important to recognize, however, that carotid sinus hypersensitivity is also commonly observed in asymptomatic elderly patients. [16] Thus, the diagnosis of carotid sinus hypersensitivity should be approached cautiously after excluding alternative causes of syncope. Once diagnosed, dualchamber pacemaker implantation is recommended for patients with recur-rent syncope or falls resulting from carotid sinus hypersensitivity. [17] [18]
Cardiac Causes of Syncope

Cardiac causes of syncope, particularly tachyarrhythmias and bradyarrhythmias, are the second most common causes, accounting for 10 to 20 percent of syncopal episodes (see Chap. 35) . Ventricular tachycardia (VT) is the most common tachyarrhythmia that can cause syncope. Supraventricular tachycardia can also cause syncope, although the great majority of patients with supraventricular arrhythmias present with less severe symptoms, such as palpitations, dyspnea, and lightheadedness. Bradyarrhythmias that can result in syncope include sick sinus syndrome as well as AV block. Anatomical causes of syncope include obstruction to blood flow, such as a massive pulmonary embolus, an atrial myxoma, or aortic stenosis.
Neurological Causes of Apparent Syncope

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Neurological causes of apparent syncope, including migraines, seizures, Arnold-Chiari malformations, and transient ischemic attacks, are surprisingly uncommon, accounting for less than 10 percent of all cases of syncope. Most patients with an apparent neurological cause of syncope have seizures rather than true syncope.
Metabolic Causes of Apparent Syncope

Metabolic causes of syncope are rare, accounting for less than 5 percent of syncopal episodes. The most common metabolic causes of syncope are hypoglycemia, hypoxia, and hyperventilation. Establishing hypoglycemia as the cause of an apparent loss of consciousness requires demonstration of hypoglycemia during the syncopal episode. Although hyperventilation-induced syncope has been generally considered to be caused by a reduction in cerebral blood flow, this remains an area of debate. Hyperventilation-induced syncope may also have a psychological component. Psychiatric disorders may also cause syncope. Up to 25 percent of patients with syncope of unknown origin may have psychiatric disorders for which apparent syncope is one of the presenting symptoms. [1]
Relationship Between Prognosis and Cause of Syncope

The prognosis for patients with syncope varies greatly with the diagnosis. Syncope of unknown origin or syncope with a noncardiac cause (including reflex-mediated syncope) is generally associated with a benign prognosis. In contrast, syncope with a cardiac cause is associated with up to 30 percent mortality at 1 year. [1] [2]

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