ReviewPostural Tachycardia Syndrome: A Heterogeneous and Multifactorial Disorder
Section snippets
Symptoms Reflecting Orthostatic Intolerance
POTS is one of the most common syndromes of orthostatic intolerance; others include reflex (neurally mediated, vasovagal) syncope and orthostatic hypotension in its several forms.3, 10 The manifestations of POTS that reflect orthostatic intolerance (POTS in the strict sense) include those of cerebral hypoperfusion and reflex sympathetic activation. Up to one-third of patients may develop secondary orthostatically triggered vasovagal (reflex, neurally mediated) syncope.11 As in all types of
Comorbidities in POTS
Many patients with POTS experience chronic symptoms that cannot be mechanistically explained by postural intolerance or excessive tachycardia.2, 37 Many of these symptoms are also prevalent in patients without orthostatic intolerance; in these cases, excessive postural tachycardia is secondary to hypovolemia, prolonged bed rest, physical deconditioning, and anxiety, in various combinations.
Potential Mechanisms of Persistence of Symptoms
The persistence of orthostatic symptoms despite adequate control of the heart rate and the coexistence of many nonorthostatic symptoms commonly reported by patients with POTS suggest that impaired processing of viscerosensory (including cardiovascular) information, conditioning, and behavioral amplification also play a contributory role in this disorder. For example, many triggers, such as viral illness (particularly if associated with gastrointestinal fluid loss), prolonged bed rest, or both,
Evaluation and Management of Patients With POTS
Patients with POTS require a multidisciplinary evaluation and multimodality treatment.2, 12
Conclusions and Perspective
POTS is a prototypical chronic, potentially disabling condition with no clear pathologic substrate and multiple interacting pathophysiologic mechanisms. Thus, it resembles functional visceral pain/dysmotility disorders, fibromyalgia, chronic headache, and chronic fatigue syndrome. In POTS, as in all these comorbid disorders, symptoms frequently develop after a triggering factor such as a viral illness or surgical procedure and persist despite resolution of the underlying condition. This
References (96)
- et al.
Postural tachycardia in children and adolescents: what is abnormal?
J Pediatr
(2012) - et al.
Postural tachycardia syndrome: clinical features and follow-up study
Mayo Clin Proc
(1999) - et al.
Postural orthostatic tachycardia syndrome: the Mayo Clinic experience
Mayo Clin Proc
(2007) - et al.
A screen of candidate genes and influence of beta2-adrenergic receptor genotypes in postural tachycardia syndrome
Auton Neurosci
(2005) - et al.
The roles of orthostatic hypotension, orthostatic tachycardia, and subnormal erythrocyte volume in the pathogenesis of the chronic fatigue syndrome
Am J Med Sci
(2000) - et al.
Blood volume perturbations in the postural tachycardia syndrome
Am J Med Sci
(2007) - et al.
Cardiac origins of the postural orthostatic tachycardia syndrome
J Am Coll Cardiol
(2010) - et al.
Comorbidities in pediatric patients with postural orthostatic tachycardia syndrome
J Pediatr
(2011) - et al.
Dysautonomia in the joint hypermobility syndrome
Am J Med
(2003) - et al.
Chronic pain is a manifestation of the Ehlers-Danlos syndrome
J Pain Symptom Manage
(1997)
Orthostatic intolerance and the headache patient
Semin Pediatr Neurol
Chiari I malformation as a cause of orthostatic intolerance symptoms: a media myth?
Am J Med
Chronic subjective dizziness
Otolaryngol Clin North Am
Quality of life in patients with postural tachycardia syndrome
Mayo Clin Proc
The science of fibromyalgia
Mayo Clin Proc
The impact of chronic primary insomnia on the heart rate—EEG variability link
Clin Neurophysiol
Dissecting axes of autonomic control in humans: insights from neuroimaging
Auton Neurosci
The emotional motor system
Prog Brain Res
Evidence for macular gravity receptor modulation of hypothalamic, limbic and autonomic nuclei
Neuroscience
Rethinking the emotional brain [published correction appears in Neuron. 2012;73(5):1052]
Neuron
Psychophysiology of neural, cognitive and affective integration: fMRI and autonomic indicants
Int J Psychophysiol
Psychopathology and the human connectome: toward a transdiagnostic model of risk for mental illness
Neuron
The pain matrix reloaded: a salience detection system for the body
Prog Neurobiol
Central nervous system reorganization in a variety of chronic pain states: a review
PM R
Postural tachycardia syndrome (POTS)
Neurology
Postural tachycardia syndrome (POTS)
J Cardiovasc Electrophysiol
Consensus statement on the definition of orthostatic hypotension, neurally mediated syncope and the postural tachycardia syndrome
Clin Auton Res
Deconditioning in patients with orthostatic intolerance [published online ahead of print September 19, 2012]
Neurology
The postural tachycardia syndrome
Cardiol Rev
A review of postural orthostatic tachycardia syndrome
Europace
Symptoms and signs of syncope: a review of the link between physiology and clinical clues
Brain
Postural tachycardia syndrome with asystole on head-up tilt
Clin Auton Res
Postural tachycardia syndrome—current experience and concepts
Nat Rev Neurol
The hemodynamic and neurohumoral phenotype of postural tachycardia syndrome
Neurology
Beta2-adrenoceptor genotype and function affect hemodynamic profile heterogeneity in postural tachycardia syndrome
Hypertension
Regional blood volume and peripheral blood flow in postural tachycardia syndrome
Am J Physiol Heart Circ Physiol
The neuropathic postural tachycardia syndrome
N Engl J Med
Pooling in chronic orthostatic intolerance: arterial vasoconstrictive but not venous compliance defects
Circulation
Cardiac neurotransmission imaging with 123I-meta-iodobenzylguanidine in postural tachycardia syndrome
J Neurol Neurosurg Psychiatry
Pathogenesis of hyperadrenergic orthostatic hypotension: evidence of disordered venous innervation exclusively in the lower limbs
J Clin Invest
The postural tachycardia syndrome (POTS): pathophysiology, diagnosis & management
Indian Pacing Electrophysiol J
Orthostatic intolerance and tachycardia associated with norepinephrine-transporter deficiency
N Engl J Med
Hyperadrenergic postural tachycardia syndrome in mast cell activation disorders
Hypertension
The protean manifestations of pheochromocytoma
Horm Metab Res
Anti-VGKC antibody-associated limbic encephalitis/Morvan syndrome [in Japanese]
Brain Nerve
Defects in cutaneous angiotensin-converting enzyme 2 and angiotensin-(1-7) production in postural tachycardia syndrome
Hypertension
Reduced stroke volume during exercise in postural tachycardia syndrome
J Appl Physiol
POTS versus deconditioning: the same or different?
Clin Auton Res
Cited by (271)
The use and effectiveness of exercise for managing postural orthostatic tachycardia syndrome in young adults with joint hypermobility and related conditions: A scoping review
2024, Autonomic Neuroscience: Basic and ClinicalPooled rates and demographics of POTS following SARS-CoV-2 infection versus COVID-19 vaccination: Systematic review and meta-analysis
2023, Autonomic Neuroscience: Basic and ClinicalEditorial commentary: Postural orthostatic tachycardia syndrome: Moving forward, not standing still
2023, Trends in Cardiovascular Medicine