Postural Orthostatic Tachycardia Syndrome a K Agarwal, R Garg, a Ritch, P Sarkar
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478 REVIEW Postural orthostatic tachycardia syndrome A K Agarwal, R Garg, A Ritch, P Sarkar ................................................................................................................................... Postgrad Med J 2007;83:478–480. doi: 10.1136/pgmj.2006.055046 Postural orthostatic tachycardia syndrome (POTS) is an concentrations, a-receptor sensitivity, b-receptor hypersensitivity5 and baroreceptor dysfunction.12 autonomic disturbance which has become better understood in Impaired innervation of the veins or their recent years. It is now thought to encompass a group of response to sympathetic stimulation plays a key disorders that have similar clinical features, such as orthostatic role in the aetiopathogenesis of POTS.13 14 This intolerance, but individual distinguishing parameters—for leads to dependent venous pooling in the legs and reduced venous return to the heart. Thus there is example, blood pressure and pulse rate. The clinical picture, redistribution of blood in the peripheral circula- diagnosis, and management of POTS are discussed. tion. This could be worsened by capillary leakage, ............................................................................. which leads to further hypovolaemia and causes reflex tachycardia. There is sympathetic cardiac activity without vasoconstriction, inducing a fall in ostural orthostatic tachycardia syndrome central venous pressure. When this process con- (POTS) is an autonomic disturbance1 char- tinues, circulatory collapse may occur. Pacterised by the clinical symptoms of ortho- static intolerance, mainly light-headedness, fatigue, sweating, tremor, anxiety, palpitation, TYPES/VARIANTS exercise intolerance and near syncope on upright POTS can be classified as primary and secondary posture.2 These are relieved on lying down. (box 1).15 Partial dysautonomia can be caused by Patients also have a heart rate .120 beats/min various stresses and has an immune mediated (bpm) on standing or increase their heart rate by pathogenesis. Serum autoantibodies to a3 acet- 30 bpm from a resting heart rate after standing for ylcholine receptors of the peripheral ganglia have 10 min. The symptoms lead to the limitation of been found. The partial dysautonomic form can activities impacting on a patient’s life on a day-to- also affect adolescents. Symptoms initially worsen day basis—for example, bathing, housework, until the patient reaches 16 years of age and then feeding. The severity of symptoms is more pro- gradually fade away. This is thought to be due to nounced compared to a condition like pure autonomic imbalance. autonomic failure. Patients with POTS may not In some primary cases there is an hyperadre- have blood pressure changes and can have nergic state16 leading to increased norepinephrine, fluctuation of blood pressure in either direction. which could be due to impaired clearance or Other conditions like chronic debilitating disor- decreased uptake by the synaptic cleft. These ders, prolonged bed rest or medication that impair variants cause profuse sweating, anxiety and autonomic function have to be excluded. tremulousness and the diastolic pressure is high. This is thought to be a genetic disorder with HISTORY AND EPIDEMIOLOGY involvement of family members. In these patients POTS was first described 1940. Low et al from the symptoms will be gradual and progressive. They Mayo clinic did the pioneering work on this will have orthostatic tachycardia as well as 3 condition. Robertson of the Vanderbilt autonomic associated hypertension. laboratories stated it was one of the most common conditions in young females.4 Frolich et al reported Evaluation patients who developed symptomatic postural A detailed history of orthostatic intolerance tachycardia without any change in blood pressure.5 (box 2), and specific findings on physical exam- The condition is common in the age group 12–50 ination (box 3) and investigations (box 4), will years with a female to male ratio of 5:1. It is give clues to the diagnosis. common after stress such as sepsis, pregnancy, Catecholamines should be measured in serum as fever, surgery or trauma.16In some of the variants well as in 24 h urine. In some variants of POTS of POTS there is a functional mutation in the gene there is a high concentration of catecholamines in See end of article for encoding for norepinephrine (noradrenaline) authors’ affiliations blood as well as urine, and a high concentration of ........................ transportation. Ala 457pro mutation renders the transfers non-functional leading to altered heart their metabolites like dihydroxyphenylglycol in Correspondence to: rate as well as epinephrine (adrenaline) metabo- urine. There is impaired response to tyramine Dr Anil K Agarwal, DGM 7 with reduced clearance of norepinephrine at the Building, City Hospital, lites. Dudley Road, Birmingham B18 7QH, UK; PATHOGENESIS Abbreviations: CDC, US Centers for Disease Control and [email protected] Several theories have been suggested for the Prevention; CFS, chronic fatigue syndrome; ME, myalgic 8–11 encephalomyelitis; NMH, neurally mediated hypotension; Received 4 November 2006 aetiopathogenesis of POTS. Suggested factors NRI, norepinephrine reuptake inhibitor; POTS, postural Accepted 20 March 2007 that may lead to POTS include impaired orthostatic tachycardia syndrome; SSRI, selective serotonin ........................ vascular innervation, high plasma norepinephrine reuptake inhibitor www.postgradmedj.com Postural tachycardia syndrome 479 Box 1 Classification of postural tachycardia Box 2 Symptoms of orthostatic intolerance syndrome N Headache Primary forms N Fatigue Partial dysautonomic Immune mediated pathogenesis N Sleep disorder Adolescence N Weakness Hyperadrenergic state N Hyperventilation/dyspnoea Secondary forms N Tremulousness Diabetes mellitus N Sweating Amyloidosis N Heavy metal poisoning Anxiety/palpitation Sjogren syndrome N Dizziness/vertigo Hypermobility syndrome N Pre-syncope/syncope Paraneoplastic syndrome enzyme inhibitors, a-andb-blockers, calcium channel blockers, synaptic cleft. Sweat test as well as skin resistance can assess diuretics, monoamine oxidase inhibitors, tricyclic antidepressants sudomotor function. and phenothiazines. Any such drugs should be stopped first. Once The 70˚ head up tilt table study has become the standard the diagnosis is confirmed aggravating factors such as dehydra- stress test for orthostatic integrity and thus of neurovascular tion, extreme heat and excess consumption of alcohol should be competence. Patterns of heart rate as well as blood pressure avoided. response will be the key to identifying which type of orthostatic intolerance is present and thus for planning treatment for Non-pharmacological measures further management. In a control population there is an Patients should be advised to take aerobic exercise on a regular increase of only 15 bpm in heart rate in the first minute of basis so that venous return from the lower extremities can be standing and a further increase for 9 min. In POTS supine heart increased.21 Patients with dysautonomic syncope can be advised rate is greater than the normal control population and an to wear graded compressive hosiery extending up to the waist, increase of more than 30 bpm takes place between 1–5 min on thus helping to increase static pressure at the calf and decrease 70˚head uplift.10 17 venous pooling. A high fluid intake should be encouraged with at least 3–5 g of common salt. DIFFERENTIAL DIAGNOSIS Pharmacotherapy Inappropriate sinus tachycardia syndrome There is no specific drug treatment for POTS and it depends on This is a clinical condition in which the heart rate increases out the type of aetiology responsible for the syndrome. of proportion to physical needs. The automaticity of the sinus Fludrocortisone has been tried with success.22 It helps by node is increased with increased cardiac sympathetic tone and increasing sodium and fluid retention as well as by sensitising blunted cardiac parasympathetic tone. Most of the features are a-adrenergic receptors. There are volume-expanding agents like similar to POTS, but in inappropriate sinus tachycardia vasopressin, and vasoconstriction drugs like midodrine, which syndrome, unlike POTS, the heart rate never exceeds 100 bpm can be gradually titrated upward until the maximum dose is in the supine position and increases on standing. reached. Midodrine is an a-1 receptor stimulating agent which helps to improve orthostatic tolerance.23 Methylphenidate, a Chronic fatigue syndrome long acting a-agonist, also has been found to be effective.24 In Europe, chronic fatigue syndrome (CFS) is called myalgic Erythropoietin, which helps in volume expansion as well encephalomyelitis (ME). Orthostatic intolerance is a broad title having a direct vasoconstrictive effect, is worth trying in for blood pressure abnormalities such as neurally mediated patients who are refractory to other treatment. The major hypotension (NMH) and POTS. Orthostatic intolerance is a disadvantages of using erythropoietin are that it is expensive symptom of CFS. CFS is a chronic condition associated with and has to be given subcutaneously.25 high morbidity. The US Centers for Disease Control and The hyperadrenergic form of POTS can be treated by b- Prevention (CDC) criteria for the diagnosis of CFS include blocking agents or drugs like labetalol, which have both a- and chronic debilitating fatigue lasting for 6 months or more, b-receptor blocking effects. In selected patients drugs such as associated