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Postural tachycardia syndrome

TOPIC OUTLINE
SUMMARY & RECOMMENDATIONS
INTRODUCTION AND TERMINOLOGY
EPIDEMIOLOGY
ETIOLOGY
Distal denervation
Hypovolemia
Changes in venous function
Baroreflex abnormalities
Increased sympathetic activity
Genetic abnormalities
Associated disorders
CLINICAL FEATURES
DIAGNOSIS
DIFFERENTIAL DIAGNOSIS
TREATMENT
SUMMARY AND RECOMMENDATIONS
REFERENCES

GRAPHICS View All

FIGURES
HR and BP response in tilt table test
TABLES
CNS causes of autonomic dysfunction
PNS causes of autonomic dysfunction

RELATED TOPICS
Clinical features and diagnosis of chronic fatigue syndrome
Clinical manifestations, pathogenesis, and classification of mastocytosis (cutaneous and systemic)
Evaluation of parasympathetic nervous system function
Mechanisms, causes, and evaluation of orthostatic and postprandial hypotension
Mitral valve prolapse syndrome
Sinus tachycardia
Treatment of orthostatic and postprandial hypotension
Postural tachycardia syndrome
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Postural tachycardia syndrome
Authors
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Roy Freeman, MD
Horacio Kaufmann, MD
Section Editor
Michael J Aminoff, MD, DSc
Deputy Editor
Janet L Wilterdink, MD
Disclosures
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Mar 2013. | This topic last updated: Feb 25, 2013.
INTRODUCTION AND TERMINOLOGY Symptomatic falls in blood pressure after standing or
eating are a frequent clinical problem, particularly in the elderly. The symptoms are often due to cerebral
hypoperfusion and include generalized weakness, dizziness or lightheadedness, visual blurring or
darkening of the visual fields, and, in severe cases, loss of consciousness. These patients often have a
decrease in sympathetic activity that leads to systemic hypotension with standing.
Another form of orthostatic intolerance occurs in patients, particularly younger adults and children, who
consistently or frequently experience symptoms of orthostatic intolerance in response to postural
stressors. Autonomic reflexes are relatively preserved in these patients, and orthostatic hypotension and
syncope rarely occur. Some patients may have slightly elevated blood pressure. The hallmark of this
disorder is an exaggerated heart rate increase in response to postural change. This disorder has been
called the postural tachycardia syndrome or POTS [ 1-3 ].
The nosology of POTS is confusing; several similar terms have been used to describe the disorder:
Chronic orthostatic intolerance
Mild orthostatic intolerance
Orthostatic tachycardia
Sympathotonic orthostatic hypotension
Hyperdynamic beta adrenergic state
Idiopathic hypovolemia
Mitral valve prolapse syndrome
Neurocirculatory asthenia
Irritable heart
Soldier's heart
Effort syndrome
This topic will review the clinical features, diagnosis, etiology, and treatment of POTS. Other causes of
orthostatic and postprandial hypotension are discussed separately. (See "Mechanisms, causes, and
evaluation of orthostatic and postprandial hypotension" and "Treatment of orthostatic and postprandial
hypotension" .)
EPIDEMIOLOGY The postural tachycardia syndrome (POTS) is the most prevalent form of
orthostatic intolerance. It is estimated that 500,000 Americans suffer from this disorder [ 4 ]. It is the most
common syndrome of young people seen in autonomic dysfunction clinics [ 5,6 ]. Patients present at a
relatively young age (14 to 45 years).
Women predominate among patients with POTS with a female to male ratio of 4-5:1 [ 3,7 ]. The reason
for this is not known, however, observed gender differences in muscle sympathetic nerve discharge
characteristics in healthy patients may explain why women are more likely to develop POTS [ 8 ].
ETIOLOGY The etiology of postural tachycardia syndrome (POTS) is heterogeneous. Investigators
have reported a number of different abnormalities in patients with POTS. It remains uncertain as to which
of these abnormalities are primary and causative and which are secondary. Some of the proposed
mechanisms discussed below are complementary.
Distal denervation Several clinical and empiric observations suggest the presence of distal,
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predominantly lower extremity, denervation with preserved cardiac innervation in this disorder. These
findings include:
Lower extremity anhidrosis [ 7,9 ]
Impaired norepinephrine spillover in the lower extremities [ 10 ], which refers to the measured rate
of entry of norepinephrine into the femoral vein in response to various stimuli: sodium
nitroprusside infusion, tyramine infusion, the cold pressor test
Decreased muscle sympathetic nerve activity recruitment in the lower extremity in response to a
nitroprusside -induced hypotensive stimulus [ 11 ]
Abnormal quantitative sudomotor axon reflex test, indicating postganglionic sympathetic
dysfunction [ 7 ]
These may result from an underlying autonomic neuropathy that may be postviral or immune-mediated in
origin [ 1,12 ]. The clinical observation that many cases originate after systemic infection lends some
support to this possibility, as does the finding of ganglionic acetylcholine receptor antibody in some
patients [ 7,13 ].
Hypovolemia Patients with POTS frequently experience symptomatic improvement with saline
infusion. Additional evidence of a decrease and/or redistribution of blood volume is observed in several
studies of patients with POTS, which have noted:
Hypovolemia [ 7,14,15 ]
Trend toward hypovolemia [ 16 ]
Reduced erythrocyte volume [ 3,17 ]
Excessive venous pooling with redistributive hypovolemia [ 18 ]
The underlying cause of the hypovolemia is uncertain. These deficits may be accompanied by impairment
of the renin-angiotensin-aldosterone system. In some investigations, despite a relative plasma volume
depletion at rest, POTS patients did not have increased plasma renin activity and did have significantly
reduced plasma aldosterone compared with controls [ 19-21 ]. These observations, combined with the
finding of reduced red blood cell volume, has led some investigators to suggest that a renal disorder,
perhaps renal denervation, may play a primary role in the etiology of POTS [ 3,20,21 ].
Alternatively, mild volume constriction may be a secondary phenomenon, the result of chronic
sympathetic activation [ 3,7 ].
Changes in venous function Abnormal venous function with decreased venous return on assumption
of the upright posture could stimulate a compensatory tachycardia in order to maintain cardiac output.
There is conflicting evidence on the changes in venous function in POTS. In some case series, military
antishock trousers have been shown to attenuate orthostatic tachycardia in the majority of patients [ 18,22
]. There are also reports of decreased stroke volume on assuming upright posture [ 2,23 ], excessive
venous pooling [ 18,24 ], and venous denervation [ 25 ] in patients with POTS. However, empiric
measurements of venous compliance have been normal [ 5 ] or decreased [ 16 ]. The latter apparently
discordant data may relate to the presence of microvascular transudation in some patients, which may
both reduce venous compliance and contribute to venous pooling [ 26 ].
Abnormal venous function may be the result of sympathetic denervation in the legs, or elevated levels of
circulating or locally released vasodilators [ 27 ].
Baroreflex abnormalities The increase in heart rate without blood pressure change upon standing in
POTS suggests a primary abnormality in baroreflex control. There is additional evidence of an attenuated
vagal baroreflex response in POTS, as measured by changes in heart rate variability in response to
valsalva and lower body negative pressure [ 16,28 ]. (See "Evaluation of parasympathetic nervous system
function" .)
Increased sympathetic activity Increased sympathetic activity is the final common pathway of most
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proposed mechanisms in POTS. Some investigators suggest that increased sympathetic activation may be
a primary etiologic factor in POTS, as suggested by the following observations in some case series:
Elevated arterial norepinephrine (NE) levels at rest [ 7,21,29 ]
Decreased NE clearance [ 21,30 ]
Increased resting heart rate in patients compared with controls [ 6,21,29 ]
An exaggerated heart rate response to isoproterenol infusion, suggestive of adrenoreceptor
hypersensitivity [ 31 ]
Larger amplitude sympathetic bursts observed in peroneal nerve recordings during rest that, in
contrast to controls, does not change during hypotension [ 8 ]
Genetic abnormalities In one large series, 12.5 percent of 152 patients with POTS reported a family
history of orthostatic intolerance [ 7 ].
In one family, a mutation was identified in the gene encoding the norepinephrine transporter, which has a
pivotal role in norepinephrine uptake at the synaptic cleft [ 32 ]. Impaired norepinephrine clearance may
contribute to excessive sympathetic activation, and the observed clinical manifestations of POTS. This
finding, while not applicable to most POTS patients, supports a primary etiologic role for excessive
sympathetic activation, at least in some patients.
Polymorphisms in genes encoding nitric oxide synthase [ 33 ] and the beta-2 adrenoreceptor [ 34 ] may
also play a role in the genesis of the postural tachycardia syndrome.
Associated disorders The chronic fatigue and the mitral valve prolapse syndromes have clinical
features which overlap with this one [ 35,36 ]. These disorders are discussed in more detail separately.
(See "Mitral valve prolapse syndrome" and "Clinical features and diagnosis of chronic fatigue syndrome"
.)
Mast cell activation abnormalities (eg, episodes of flushing associated with abnormal increases in urine
methylhistamine) have also been noted in some patients with POTS [ 36 ]. Vasoactive compounds such as
histamine are released in mast cell activation and may contribute to clinical manifestations. (See "Clinical
manifestations, pathogenesis, and classification of mastocytosis (cutaneous and systemic)", section on
'Clinical features' .) Alternatively, evidence of mast cell activation in POTS may be a secondary (stressinduced) phenomenon.
POTS has also been associated with Ehlers-Danlos syndrome and joint hypermobility disorders [ 37-39 ].
CLINICAL FEATURES Patients with postural tachycardia syndrome (POTS) report dizziness,
lightheadedness, weakness, blurred vision, and fatigue upon standing [ 7,13 ]. Other predominantly
orthostatic symptoms include palpitations, tremulousness, and anxiety. Gastrointestinal symptoms such as
nausea, abdominal cramps, early satiety, bloating, constipation, and diarrhea may be particularly
problematic in some. There may also be evidence of venous pooling, as manifested by acrocyanosis and
edema when upright [ 3 ]. Syncope is relatively unusual, but does occur in about 40 percent of patients [
40 ].
The pathogenesis of the symptoms in POTS is largely unelucidated. Interventions that successfully
attenuate the tachycardia do not always bring symptomatic relief [ 22 ]. Some of the symptoms suggest
cerebral hypoperfusion despite normal systemic blood pressure. However, there is no definitive evidence
of abnormal cerebral blood flow or deficient cerebral autoregulation in patients with POTS [ 22,41 ].
The symptoms may appear abruptly, often after a viral illness; others experience a more insidious onset [
7,13 ]. The severity of symptoms is also quite variable. Some patients experience only mild symptoms
and often only in the setting of additional orthostatic stress (eg, menstrual cycle, relative dehydration).
Others are profoundly incapacitated. The course of the disorder may be self limited or may follow a
relapsing remitting course over several years.
DIAGNOSIS The characteristic autonomic abnormality in patients with postural tachycardia
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syndrome (POTS) is an exaggerated increase in heart rate on tilt table testing or standing.
Diagnostic criteria from several laboratories have in common a sustained heart rate increase of greater
than 30 beats per minute or an increase to 120 beats per minute or greater within the first 10 minutes of
tilt [ 1-3,10,13,24,28,42-44 ]. There is usually no orthostatic hypotension ( figure 1 )[ 45 ].
Different criteria might be more appropriate in children and adolescents. In one series of children 8 to 19
years old, many normal control children were found to have the changes described above in adults [ 46 ].
In this series, criteria that discriminated between patients and controls were: for children <14 years, a
sustained heart rate increase of greater than 40 beats per minute or an increase to 130 beats per minute or
greater within the first five minutes of tilt, and for children 14 to 19 years, a sustained heart rate increase
of greater than 40 beats per minute or an increase to 120 beats per minute or greater within the first five
minutes of tilt.
These abnormalities may be accompanied by an increase in venous plasma norepinephrine levels at rest
and upon standing (>600 ng/mL) [ 1,6,10 ].
DIFFERENTIAL DIAGNOSIS Autonomic neuropathies, central dysautonomias, bedrest
deconditioning, side effects of medications, and dehydration can produce similar symptoms to POTS (
table 1 and table 2 ). Ruling out these conditions is essential to making a diagnosis of POTS. (See
"Mechanisms, causes, and evaluation of orthostatic and postprandial hypotension" .) In most cases,
historical information and a neurologic examination specifically looking for other evidence of autonomic
failure, neuropathy, and extrapyramidal signs, will provide evidence of the underlying disorder.
Patients with POTS may be thought to have panic, anxiety, somatization disorder, or chronic fatigue
syndrome in part because of the vague nature of the symptoms. In fact, patients with POTS report
subjective cognitive dysfunction and have objectively increased scores on inattention scales, but do not
have an increased prevalence of depression or anxiety [ 47 ]. The prominent postural nature of the
symptoms should prompt the clinician to look for the diagnostic heart rate response.
The syndrome of inappropriate sinus tachycardia is characterized by an elevated heart rate that is not
influenced by postural changes. This disorder is discussed separately. (See "Sinus tachycardia", section
on 'Inappropriate sinus tachycardia' .)
TREATMENT The optimal therapy of postural tachycardia syndrome (POTS) is uncertain. No
intervention has been systematically studied. The placebo effect may be substantial in POTS, highlighting
the need for controlled studies [ 48 ]. Exacerbating factors, medications, dehydration, and inactivity
should be avoided.
Because many patients with POTS have a low plasma volume, correction with oral volume expansion, a
high salt diet, and fludrocortisone , a mineralocorticoid agonist may improve symptoms [ 45 ]. This
regimen is similar to that used in orthostatic hypotension in general. (See "Treatment of orthostatic and
postprandial hypotension", section on 'Increased salt and water intake' and "Treatment of orthostatic and
postprandial hypotension", section on 'Fludrocortisone' .)
Some patients report symptomatic benefit with acute ingestion of 16 oz of water and from a saline
infusion of 500 to 2000 cc, corresponding to objective improvement in tilt testing response [ 3,15,49 ].
However, it is not clear that this translates to a therapeutic response to chronic treatment. Fludrocortisone
(0.1 to 0.4 mg per day) is most effective when combined with increased salt and water intake. Treatment
may be complicated by supine hypertension, fluid retention, and hypokalemia and should be monitored
closely.
Adrenoreceptor agonists may be helpful in some patients (eg, midodrine 2.5 to 10 mg three times daily).
Both intravenous phenylephrine and oral midodrine have been associated with improved symptoms and
heart rate response in some patients during tilt testing [ 6,15,50 ]. However, benefit from chronic therapy
is not established.
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Preliminary evidence suggests that the acetylcholinesterase inhibitor pyridostigmine (30 mg daily) may
attenuate the tachycardia and improve symptoms [ 48 ]. Further confirmation from larger trials is needed
to establish the benefit of acetylcholinesterase inhibition for POTS.
Some patients, particularly those troubled by prominent adrenergic symptoms, may benefit from beta
blocking agents. These should be started in low doses and increased gradually (eg, propranolol 20 to 30
mg three or four times daily). In one placebo-controlled, randomized crossover study, a single low-dose
of oral propranolol (20 mg) was associated with improved tachycardia and reduced symptoms, while
higher dose propranolol (80 mg) was associated with unchanged or worsened symptoms [ 51 ]. As with
other agents, positive effects on symptoms and tilt testing response in the short term have been reported
with propranolol, but long term benefit remains uncertain [ 31,50 ].
Clonidine , methyldopa , phenobarbital , erythropoietin, and selective serotonin reuptake inhibitors
(SSRIs) are used rarely in POTS [ 7,15,52 ]. Sinus node ablation is not effective; although the sinus rate is
effectively slowed, there is no significant improvement in clinical symptoms [ 44 ]. (See "Sinus
tachycardia", section on 'Inappropriate sinus tachycardia' .)
SUMMARY AND RECOMMENDATIONS
The postural tachycardia syndrome (POTS) is defined as a form of orthostatic intolerance
characterized by an excessive increase in heart rate that occurs on standing without arterial
hypotension (see 'Introduction and terminology' above).
The etiology of POTS is not clear, but the disorder may be heterogeneous. Abnormalities in
autonomic regulation that may either be genetic or acquired are described. Proposed mechanisms
include partial sympathetic denervation leading to discordant cardiac and vascular sympathetic
control, hypovolemia and impairment of the renin-angiotensin-aldosterone system, venous
abnormalities and baroreflex dysfunction (see 'Etiology' above).
The clinical symptoms of POTS are varied and nonspecific, and include dizziness, lightheadedness,
weakness, blurred vision, and fatigue upon standing. The orthostatic nature of the symptoms is the
primary clue to the diagnosis. (See 'Clinical features' above.)
The diagnosis of POTS is established from the history and head-up tilt testing which demonstrates
a heart rate increase of >30 bpm over baseline or to >120 bpm. Dehydration, prolonged bedrest,
medications, and other dysautonomias should be excluded as etiologies (see 'Diagnosis' above and
'Differential diagnosis' above).
The optimal therapy of POTS is not established. We suggest volume repletion and fludrocortisone
(0.1 to 0.4 mg per day) as the first line of therapy ( Grade 2C ). Some patients may benefit from
midodrine or beta blocking agents. Other therapies remain under investigation, and further
confirmation of benefit is needed before they can be recommended (see 'Treatment' above).
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