Postural orthostatic tachycardia syndrome (
POTS, also
postural tachycardia syndrome) is a condition of
dysautonomiaDysautonomia is a broad term that describes any disease or malfunction of the autonomic nervous system. This includes postural orthostatic tachycardia syndrome , inappropriate sinus tachycardia , vasovagal syncope, mitral valve prolapse dysautonomia, pure autonomic failure, neurocardiogenic...
, more specifically
orthostatic intoleranceOrthostatic intolerance is a subcategory of dysautonomia, a disorder of the autonomic nervous system occurring when an individual stands up....
, in which a change from the
supine positionThe supine position is a position of the body: lying down with the face up, as opposed to the prone position, which is face down, sometimes with the hands behind the head or neck. When used in surgical procedures, it allows access to the peritoneal, thoracic and pericardial regions; as well as the...
to an upright position causes an abnormally large increase in heart rate, called
tachycardiaTachycardia comes from the Greek words tachys and kardia . Tachycardia typically refers to a heart rate that exceeds the normal range for a resting heart rate...
. Several studies show a decrease in cerebral blood flow with systolic and diastolic
cerebral blood flowCerebral blood flow, or CBF, is the blood supply to the brain in a given time. In an adult, CBF is typically 750 millitres per minute or 15% of the cardiac output. This equates to 50 to 54 millilitres of blood per 100 grams of brain tissue per minute. CBF is tightly regulated to meet the brain's...
(CBF) velocity decreased 44% and 60%, respectively. Patients with POTS have problems maintaining homeostasis when changing position, i.e. moving from one chair to another or reaching above their heads. Many patients also experience symptoms when stationary or even while lying down.
Symptoms present in various degrees of severity depending on the patient. POTS can be severely debilitating. Some patients are unable to attend school or work, and especially severe cases can completely incapacitate the patient.
Symptoms
The hallmark symptom of POTS is an increase in heart rate from the supine to upright position of more than 30 beats per minute or to a heart rate greater than 120 beats per minute within 12 minutes of head-up tilt.
This tachycardic response is sometimes accompanied by a decrease in blood pressure and a wide variety of symptoms associated with hypotension. Low blood pressure of any kind may promote the following:
- lightheadedness
Light-headedness is a common and often unpleasant sensation of dizziness and/or feeling that one may be about to faint, which may be transient, recurrent, or occasionally chronic. In some cases, the individual may feel as though his or her head is weightless. The individual may also feel as...
, sometimes called pre-syncope (pre-fainting) dizzinessDizziness refers to an impairment in spatial perception and stability. The term is somewhat imprecise. It can be used to mean vertigo, presyncope, disequilibrium, or a non-specific feeling such as giddiness or foolishness....
(but not vertigoVertigo is a type of dizziness, where there is a feeling of motion when one is stationary. The symptoms are due to a dysfunction of the vestibular system in the inner ear...
, which is also called dizziness)
- exercise intolerance
- extreme fatigue
Fatigue is a state of awareness describing a range of afflictions, usually associated with physical and/or mental weakness, though varying from a general state of lethargy to a specific work-induced burning sensation within one's muscles...
- syncope
Syncope , the medical term for fainting, is precisely defined as a transient loss of consciousness and postural tone characterized by rapid onset, short duration, and spontaneous recovery due to global cerebral hypoperfusion that most often results from hypotension.Many forms of syncope are...
(fainting)
- Excessive thirst (polydipsia
Polydipsia is a medical symptom in which the patient displays excessive thirst. The word derives from the Greek πολυδιψία, which is derived from πολύς + δίψα...
)
Chronic or acute hypoperfusion of tissues and organs in the upper parts of the body are thought to cause the following symptoms:
- cold extremities
- chest pain
Chest pain may be a symptom of a number of serious conditions and is generally considered a medical emergency. Even though it may be determined that the pain is non-cardiac in origin, this is often a diagnosis of exclusion made after ruling out more serious causes of the pain.-Differential...
and discomfort
- disorientation
- tinnitus
Tinnitus |ringing]]") is the perception of sound within the human ear in the absence of corresponding external sound.Tinnitus is not a disease, but a symptom that can result from a wide range of underlying causes: abnormally loud sounds in the ear canal for even the briefest period , ear...
- dyspnea
Dyspnea , shortness of breath , or air hunger, is the subjective symptom of breathlessness.It is a normal symptom of heavy exertion but becomes pathological if it occurs in unexpected situations...
- headache
A headache or cephalalgia is pain anywhere in the region of the head or neck. It can be a symptom of a number of different conditions of the head and neck. The brain tissue itself is not sensitive to pain because it lacks pain receptors. Rather, the pain is caused by disturbance of the...
- muscle weakness
Muscle weakness or myasthenia is a lack of muscle strength. The causes are many and can be divided into conditions that have true or perceived muscle weakness...
- tremulousness
- visual disturbances
Autonomic dysfunctionDysautonomia is a broad term that describes any disease or malfunction of the autonomic nervous system. This includes postural orthostatic tachycardia syndrome , inappropriate sinus tachycardia , vasovagal syncope, mitral valve prolapse dysautonomia, pure autonomic failure, neurocardiogenic...
is thought to cause additional gastrointestinal symptoms:
- abdominal pain
Abdominal pain can be one of the symptoms associated with transient disorders or serious disease. Making a definitive diagnosis of the cause of abdominal pain can be difficult, because many diseases can result in this symptom. Abdominal pain is a common problem...
or discomfort
- bloating
Bloating is any abnormal general swelling, or increase in diameter of the abdominal area. As a symptom, the patient feels a full and tight abdomen, which may cause abdominal pain sometimes accompanied by increased borborygmus or more seriously the total lack of borborygmus.-Symptoms:The most common...
- constipation
Constipation refers to bowel movements that are infrequent or hard to pass. Constipation is a common cause of painful defecation...
- diarrhea
Diarrhea , also spelled diarrhoea, is the condition of having three or more loose or liquid bowel movements per day. It is a common cause of death in developing countries and the second most common cause of infant deaths worldwide. The loss of fluids through diarrhea can cause dehydration and...
- nausea
Nausea , is a sensation of unease and discomfort in the upper stomach with an involuntary urge to vomit. It often, but not always, precedes vomiting...
- vomiting
Vomiting is the forceful expulsion of the contents of one's stomach through the mouth and sometimes the nose...
Cerebral hypoperfusion, when present, can cause cognitive and emotive difficulties. Symptoms that persist in the supine (recumbent) state are difficult to attribute to "cerebral hypoperfusion"
- brain fog
- burnout
Burnout is a psychological term for the experience of long-term exhaustion and diminished interest. Research indicates general practitioners have the highest proportion of burnout cases; according to a recent Dutch study in Psychological Reports, no less than 40% of these experienced high levels of...
- decreased mental stamina
- depression
Depression is a state of low mood and aversion to activity that can affect a person's thoughts, behaviour, feelings and physical well-being. Depressed people may feel sad, anxious, empty, hopeless, helpless, worthless, guilty, irritable, or restless...
- difficulty finding the right word
- impaired concentration
- sleep disorders
Inappropriate levels of
epinephrineEpinephrine is a hormone and a neurotransmitter. It increases heart rate, constricts blood vessels, dilates air passages and participates in the fight-or-flight response of the sympathetic nervous system. In chemical terms, adrenaline is one of a group of monoamines called the catecholamines...
and
norepinephrineNorepinephrine is the US name for noradrenaline , a catecholamine with multiple roles including as a hormone and a neurotransmitter...
lead to anxiety-like symptoms:
- chills
- feelings of fear
- flushing
For a person to flush is to become markedly red in the face and often other areas of the skin, from various physiological conditions. Flushing is generally distinguished, despite a close physiological relation between them, from blushing, which is milder, generally restricted to the face, cheeks or...
- overheating
Hyperthermia is an elevated body temperature due to failed thermoregulation. Hyperthermia occurs when the body produces or absorbs more heat than it can dissipate...
- nervousness
Anxiety is a psychological and physiological state characterized by somatic, emotional, cognitive, and behavioral components. The root meaning of the word anxiety is 'to vex or trouble'; in either presence or absence of psychological stress, anxiety can create feelings of fear, worry, uneasiness,...
- over-stimulation
Symptoms of POTS overlap considerably with those of generalized anxiety disorder, and a misdiagnosis of an anxiety disorder is not uncommon.
Prolonged inactivity of any cause, especially when much time is being spent in the supine (recumbent) position, will lead to a diminution in the usual orthostatic reflex. In other words, chronic inactivity with frequent assumption of the supine position will lead to the hallmark signs of POTS. This raises the question as to whether, in some cases, POTS is actually an epiphenomenon, and has resulted from prolonged inactivity. Since many POTS sufferers feel compelled to remain supine, the potential for a vicious cycle is obvious.
Symptoms of POTS overlap with B12 deficiency in absence of anemia (folic acid fortification will correct the anemia once seen in B12 deficiency.) B12 deficiency cannot be ruled out with a complete blood count. Test B12 levels and the metabolites homocysteine and methylmalonic acid. http://www.aafp.org/afp/2003/0301/p979.html
Associated conditions
- POTS is often accompanied by vasovagal syncope
A vasovagal episode or vasovagal response or vasovagal attack is a malaise mediated by the vagus nerve. When it leads to syncope or "fainting", it is called a vasovagal syncope, which is the most common type of fainting.There are a number of different syncope syndromes which all fall under the...
, also called "neurally mediated hypotension" (NMH) or "neurocardiogenic syncope" (NCS). Vasovagal syncope is a fainting reflex due to a profound drop in blood pressure. Autonomic dysfunction that occurs with these disorders causes blood to inappropriately pool in the limbs away from the heart, lungs, and brain. The combination of misdirected bloodflow and hypotension will invoke syncope. Tachycardia associated with POTS may be a cardiac response to restore cerebral perfusion.
- POTS may be a cause of chronic fatigue syndrome
Chronic fatigue syndrome is the most common name used to designate a significantly debilitating medical disorder or group of disorders generally defined by persistent fatigue accompanied by other specific symptoms for a minimum of six months, not due to ongoing exertion, not substantially...
in patients that exhibit signs of orthostatic intoleranceOrthostatic intolerance is a subcategory of dysautonomia, a disorder of the autonomic nervous system occurring when an individual stands up....
. Treating POTS will greatly improve or even eliminate disabling fatigue for these patients.
- Some patients with fibromyalgia
Fibromyalgia is a medical disorder characterized by chronic widespread pain and allodynia, a heightened and painful response to pressure. It is an example of a diagnosis of exclusion...
complain of dysautonomia-related symptoms. Treating these patients for POTS will often improve myofascial and neuropathic pain.
- Autonomic dysfunction is most likely responsible for irritable bowel syndrome
Irritable bowel syndrome is a diagnosis of exclusion. It is a functional bowel disorder characterized by chronic abdominal pain, discomfort, bloating, and alteration of bowel habits in the absence of any detectable organic cause. In some cases, the symptoms are relieved by bowel movements...
in many patients as well.
- Patients with Ehlers-Danlos syndrome
Ehlers–Danlos syndrome is a group of inherited connective tissue disorders, caused by a defect in the synthesis of collagen . The collagen in connective tissue helps tissues to resist deformation...
may also have POTS. Joint hypermobilityHypermobility describes joints that stretch farther than is normal. For example, some hypermobile people can bend their thumbs backwards to their wrists, bend their knee joints backwards, put their leg behind the head or other contortionist performances...
is a feature of the most common subtype of Ehlers-Danlos.
- Some POTS patients experience symptoms associated with restless leg syndrome. Treating POTS should also relieve these symptoms.
- Some findings link this with hypermobility
Hypermobility describes joints that stretch farther than is normal. For example, some hypermobile people can bend their thumbs backwards to their wrists, bend their knee joints backwards, put their leg behind the head or other contortionist performances...
- A small percentage of pediatric cases of POTS have been associated with moderate to severe memory loss
Memory loss can be partial or total and it is normal when it comes with aging. Sudden memory loss is usually a result of brain trauma and it may be permanent or temporary. When it is caused by medical conditions such as Alzheimers, the memory loss is gradual and tends to be permanent.Brain trauma...
.
Causes
The causes of POTS are not fully known. Most patients develop symptoms in their teenage years during a period of rapid growth and see gradual improvement into their mid-twenties. Others develop POTS after a viral or bacterial infection such as
mononucleosisInfectious mononucleosis is an infectious, widespread viral...
or
pneumoniaPneumonia is an inflammatory condition of the lung—especially affecting the microscopic air sacs —associated with fever, chest symptoms, and a lack of air space on a chest X-ray. Pneumonia is typically caused by an infection but there are a number of other causes...
. Some patients develop symptoms after experiencing some sort of trauma such as a car accident or injury. Women can also develop POTS during or after
pregnancyPregnancy refers to the fertilization and development of one or more offspring, known as a fetus or embryo, in a woman's uterus. In a pregnancy, there can be multiple gestations, as in the case of twins or triplets...
. These patients generally have a poorer prognosis.
In one large test, 12.5% of 152 patients with POTS reported a family history of orthostatic intolerance, suggesting that there is a genetic inheritance associated with POTS.
So far no one has provided an explanation for POTS which is applicable to all sufferers, however there are many theories;
Alpha-receptor dysfunction may be occurring in some POTS patients. Alpha-1 receptors cause peripheral vasoconstriction when stimulated. Alpha-1 receptor supersensitivity may be causing
dysautonomiaDysautonomia is a broad term that describes any disease or malfunction of the autonomic nervous system. This includes postural orthostatic tachycardia syndrome , inappropriate sinus tachycardia , vasovagal syncope, mitral valve prolapse dysautonomia, pure autonomic failure, neurocardiogenic...
in some patients.
Beta-receptor supersensitivity may occur with hyperadrenergic states in some people with POTS.
Hyperdopaminergic states may be the underlying problem for some people with
orthostatic intoleranceOrthostatic intolerance is a subcategory of dysautonomia, a disorder of the autonomic nervous system occurring when an individual stands up....
. Some patients have been found to have a significant increase in upright
dopamineDopamine is a catecholamine neurotransmitter present in a wide variety of animals, including both vertebrates and invertebrates. In the brain, this substituted phenethylamine functions as a neurotransmitter, activating the five known types of dopamine receptors—D1, D2, D3, D4, and D5—and their...
levels. Free plasma norepinephrine also tends to be higher in these patients.
Reduced venous return is one of the main mechanisms that causes POTS symptoms. Venous return can be reduced due to conditions such as
hypovolemiaIn physiology and medicine, hypovolemia is a state of decreased blood volume; more specifically, decrease in volume of blood plasma...
(low plasma volume/low blood volume), venous pooling and denervation. A hyperadrenergic state may result as the body attempts to compensate for these abnormalities.
Sympathetic Overactivity is observed in many POTS patients. The sympathetic overactivity can be secondary to a number of factors, some of which may be peripheral denervation, venous pooling, or end-organ dysfunction. Sympathetic underactivity can also occur in some forms of orthostatic intolerance, such as pure autonomic failure.
Recent studies have described a subset of POTS patients that appear to have elevated angiotensin II levels coupled with paradoxically reduced absolute blood volume, signs of increased sympathetic activity and reduced peripheral blood flow. This subset of POTS patients appear to have abnormal
catabolismCatabolism is the set of metabolic pathways that break down molecules into smaller units and release energy. In catabolism, large molecules such as polysaccharides, lipids, nucleic acids and proteins are broken down into smaller units such as monosaccharides, fatty acids, nucleotides, and amino...
of Angiotensin II that may contribute to reduced blood volume and orthostatic intolerance.
Diagnosis
POTS can be difficult to diagnose. A routine physical examination and standard blood tests will not indicate POTS. A
tilt table testA tilt table test, occasionally called upright tilt testing, is a medical procedure often used to diagnose dysautonomia or syncope. Patients with symptoms of dizziness or lightheadedness, with or without a loss of consciousness , suspected to be associated with a drop in blood pressure or...
is vital to diagnosing POTS, although all symptoms must be considered before a final diagnosis is made. Tests to rule out
Addison's DiseaseAddison’s disease is a rare, chronic endocrine disorder in which the adrenal glands do not produce sufficient steroid hormones...
,
pheochromocytomaA pheochromocytoma or phaeochromocytoma is a neuroendocrine tumor of the medulla of the adrenal glands , or extra-adrenal chromaffin tissue that failed to involute after birth and secretes excessive amounts of catecholamines, usually noradrenaline , and adrenaline to a lesser extent...
, electrolyte imbalance,
Lyme DiseaseLyme disease, or Lyme borreliosis, is an emerging infectious disease caused by at least three species of bacteria belonging to the genus Borrelia. Borrelia burgdorferi sensu stricto is the main cause of Lyme disease in the United States, whereas Borrelia afzelii and Borrelia garinii cause most...
, Celiac Disease, and various food allergies are usually performed. A blood test may be performed to verify abnormally high levels of
norepinephrineNorepinephrine is the US name for noradrenaline , a catecholamine with multiple roles including as a hormone and a neurotransmitter...
present in some POTS patients.
Between 75 and 80 percent of POTS patients are female and of the menstruating age. Most male patients develop POTS in their early to mid-teens during a growth spurt or following a viral or bacterial infection. Some women also develop POTS symptoms during or after
pregnancyPregnancy refers to the fertilization and development of one or more offspring, known as a fetus or embryo, in a woman's uterus. In a pregnancy, there can be multiple gestations, as in the case of twins or triplets...
.
The timing of POTS becoming a named syndrome with no known cause coincides with the advent of folic acid fortification of foods and vitamins. Because the symptoms are the same symptoms as B12 deficiency in absence of anemia, and because the majority of the patients diagnosed with POTS have risk factors for new onset B12 deficiency (menstruating females, during a growth spurt, during or after pregancy) it is possible that POTS is a misdiagnosed B12 deficiency ("masked" by folic acid fortification, these patients no longer become anemic.) B12 deficiency must always be ruled out with any autonomous nervous system dysfunction. Failure to treat B12 deficiency with repletion of B12 may lead to irreversible damage. http://www.aafp.org/afp/2003/0301/p979.html
Prognosis
Most POTS patients will see symptom improvement over the course of several years. Those who develop POTS in their early to mid teens during a period of rapid growth will most likely see complete symptom resolution by their mid twenties. Patients with post-viral POTS will also usually improve greatly or see a full symptom resolution. Adults who develop POTS, especially women during or after pregnancy, usually see milder improvement and can be plagued with their condition for life. Rarely, a teenager who develops POTS will gradually worsen over time and have lifelong symptoms. Patients with secondary POTS as a consequence of
Ehlers-Danlos SyndromeEhlers–Danlos syndrome is a group of inherited connective tissue disorders, caused by a defect in the synthesis of collagen . The collagen in connective tissue helps tissues to resist deformation...
will also usually struggle with symptoms for life. In some patients the only cure for POTS is time.
Recovered individuals do complain of occasional, non-debilitating recurrence of symptoms associated with autonomic dysfunction including dizzy spells, lightheadedness, flushing, transient syncope, and symptoms of irritable bowel syndrome. These symptoms are consistent with B12 deficiency in absence of anemia, which should always be ruled out directly by checking B12, homocysteine and methylmalonic acid. http://www.aafp.org/afp/2003/0301/p979.html
Treatment
Most patients will respond to some form of treatment. Lifestyle changes, particularly drinking extra water and avoiding trigger situations such as standing still or getting hot, are necessary for all patients. Some patients also benefit from the addition of other treatments, such as certain medications.
Dietary changes
- Drinking more water improves symptoms for nearly all patients. Most patients are encouraged to drink at least 64 ounces (two liters) of water or other fluids each day.
- Alcohol
An alcoholic beverage is a drink containing ethanol, commonly known as alcohol. Alcoholic beverages are divided into three general classes: beers, wines, and spirits. They are legally consumed in most countries, and over 100 countries have laws regulating their production, sale, and consumption...
has been shown to drastically exacerbate all types of orthostatic intoleranceOrthostatic intolerance is a subcategory of dysautonomia, a disorder of the autonomic nervous system occurring when an individual stands up....
due to its vasodilationVasodilation refers to the widening of blood vessels resulting from relaxation of smooth muscle cells within the vessel walls, particularly in the large arteries, smaller arterioles and large veins. The process is essentially the opposite of vasoconstriction, or the narrowing of blood vessels. When...
and dehydrationIn physiology and medicine, dehydration is defined as the excessive loss of body fluid. It is literally the removal of water from an object; however, in physiological terms, it entails a deficiency of fluid within an organism...
properties. In addition to its adverse effects, it interacts unfavorably with many of the medications prescribed for POTS patients.
- Eating frequent, small meals can reduce gastrointestinal symptoms associated with POTS by requiring the diversion of less blood to the abdomen.
- Increasing salt intake, by adding salt to food, taking salt tablets, or drinking sports drinks and other electrolyte solutions (most doctors recommend drinking Gatorade
Gatorade is a brand of sports-themed food and beverage products, built around its signature product: a line of sports drinks. Gatorade is currently manufactured by PepsiCo, distributed in over 80 countries...
or PedialytePedialyte is an oral electrolyte solution manufactured by Abbott Laboratories and invented by Gary Cohen, MD of Swampscott, Massachusetts, which is designed to replace fluids and minerals that are lost when a child has diarrhea with or without vomiting...
, or Nuun active hydration tablets), is a treatment used for many people with POTS; however, salt is not recommended for all patients. Increasing salt is an effective way to raise blood pressure in many patients with orthostatic hypotensionOrthostatic hypotension, also known as postural hypotension, orthostasis, and colloquially as head rush or dizzy spell, is a form of hypotension in which a person's blood pressure suddenly falls when the person stands up or stretches. The decrease is typically greater than 20/10 mm Hg, and may be...
by helping the body retain water and thereby expanding blood volume. Different physicians recommend different amounts of sodium to their patients.
- Diets high in carbohydrates have been connected to impaired vasoconstrictive action. Eating foods with lower carbohydrate levels can mildly improve POTS symptoms.
- Caffeine
Caffeine is a bitter, white crystalline xanthine alkaloid that acts as a stimulant drug. Caffeine is found in varying quantities in the seeds, leaves, and fruit of some plants, where it acts as a natural pesticide that paralyzes and kills certain insects feeding on the plants...
helps some POTS patients due to its stimulative effects; however, other patients report a worsening of symptoms with caffeine intake.
- Tilting of the head of the bed to an angle of roughly 30 degrees can also help reduce symptoms.
Physical therapy and exercise
Exercise is very important for maintaining muscle strength and avoiding
deconditioningDeconditioning is adaptation of an organism to less demanding environment, or, alternatively, the decrease of physiological adaptation to normal conditions...
. Though many POTS patients report difficulty exercising, some form of exercise is essential to controlling symptoms and eventually, improving the condition. Exercises that improve leg and abdominal strength may aid in improving the muscle pump and therefore preventing pooling of blood in the abdomen and lower extremities.
Aerobic exerciseAerobic exercise is physical exercise of relatively low intensity that depends primarily on the aerobic energy-generating process. Aerobic literally means "living in air", and refers to the use of oxygen to adequately meet energy demands during exercise via aerobic metabolism...
performed for 20 minutes a day, three times a week, is sometimes recommended for patients who can tolerate it. Certain modalities of exercise may be more tolerable initially, such as riding a
recumbent bicycleA recumbent bicycle is a bicycle that places the rider in a laid-back reclining position. Most recumbent riders choose this type of design for ergonomic reasons; the rider's weight is distributed comfortably over a larger area, supported by back and buttocks...
or swimming. However, as tolerable, upright exercise may benefit the participant through orthostatic training. All exercise programs for POTS patients should begin with low intensity exercises for a short duration and progress slowly.
Medications
Patients whose POTS symptoms are due to B12 deficiency need pharmacological doses of B12 for repletion of tissues. http://www.aafp.org/afp/2003/0301/p979.html
Several classes of drugs often provide symptom control and relief for POTS patients. Treatments must be carefully tested due to medication sensitivity often associated with POTS patients, and each patient will respond to different therapies in different ways.
The first line of treatment for POTS is usually
fludrocortisoneFludrocortisone is a synthetic corticosteroid with moderate glucocorticoid potency and much greater mineralocorticoid potency. The brand name in the U.S. and Canada is Florinef.-Uses:...
, or Florinef, a mineralcorticoid used to increase sodium retention and thus increase blood volume and blood pressure. An increase in sodium and water intake must coincide with fludrocortisone therapy for effective treatment.
Dietary increases in sodium and sodium supplements are often used.
Beta blockers such as
atenolol,
metoprololMetoprolol is a selective β1 receptor blocker used in treatment of several diseases of the cardiovascular system, especially hypertension. The active substance metoprolol is employed either as metoprolol succinate or metoprolol tartrate...
and propanolol are often prescribed to treat POTS. These medications slow down the excessive heart rate response (tachycardia) that POTS patients experience. They also work by blocking the effects of
epinephrineEpinephrine is a hormone and a neurotransmitter. It increases heart rate, constricts blood vessels, dilates air passages and participates in the fight-or-flight response of the sympathetic nervous system. In chemical terms, adrenaline is one of a group of monoamines called the catecholamines...
and
norepinephrineNorepinephrine is the US name for noradrenaline , a catecholamine with multiple roles including as a hormone and a neurotransmitter...
released by the Autonomic Nervous System. In addition,Beta blockers reduce Sympathetic Nervous System activity by blocking Sympathetic impulses. For some patients, Beta blockers increase POTS symptoms (e.g. lowering blood pressure, which is why they are often prescribed in conjunction with
MidodrineMidodrine is a vasopressor/antihypotensive agent. Midodrine was approved in the United States by the Food and Drug Administration in 1996 for the treatment of orthostatic hypotension...
). Beta blockers may be dangerous to individuals with asthma or allergies.
MidodrineMidodrine is a vasopressor/antihypotensive agent. Midodrine was approved in the United States by the Food and Drug Administration in 1996 for the treatment of orthostatic hypotension...
(Proamatine), is approved by the U.S. Food and Drug Administration (FDA) to treat
orthostatic hypotensionOrthostatic hypotension, also known as postural hypotension, orthostasis, and colloquially as head rush or dizzy spell, is a form of hypotension in which a person's blood pressure suddenly falls when the person stands up or stretches. The decrease is typically greater than 20/10 mm Hg, and may be...
, a condition related to POTS. It is a stimulant that causes
vasoconstrictionVasoconstriction is the narrowing of the blood vessels resulting from contraction of the muscular wall of the vessels, particularly the large arteries, small arterioles and veins. The process is the opposite of vasodilation, the widening of blood vessels. The process is particularly important in...
and thereby increases
blood pressureBlood pressure is the pressure exerted by circulating blood upon the walls of blood vessels, and is one of the principal vital signs. When used without further specification, "blood pressure" usually refers to the arterial pressure of the systemic circulation. During each heartbeat, BP varies...
and allows more blood to return to the upper parts of the body. Use of midodrine is often discontinued due to intolerable side-effects, and it is known to cause supine hypertension (high blood pressure when lying down). Some doctors prefer to start patients on
MidodrineMidodrine is a vasopressor/antihypotensive agent. Midodrine was approved in the United States by the Food and Drug Administration in 1996 for the treatment of orthostatic hypotension...
without the concomitant use of Beta blockers and then add Beta blockers once the dose of
MidodrineMidodrine is a vasopressor/antihypotensive agent. Midodrine was approved in the United States by the Food and Drug Administration in 1996 for the treatment of orthostatic hypotension...
has been properly adjusted. This gives the
MidodrineMidodrine is a vasopressor/antihypotensive agent. Midodrine was approved in the United States by the Food and Drug Administration in 1996 for the treatment of orthostatic hypotension...
time to start raising the patient's blood pressure which often helps avoid the hypotension that is a common side effect of Beta blockers. Obviously lowering the blood pressure of a POTS patient would exacerbate any existing
orthostatic hypotensionOrthostatic hypotension, also known as postural hypotension, orthostasis, and colloquially as head rush or dizzy spell, is a form of hypotension in which a person's blood pressure suddenly falls when the person stands up or stretches. The decrease is typically greater than 20/10 mm Hg, and may be...
.
Antidepressants, especially selective serotonin reuptake inhibitors (SSRIs) such as
fluoxetineFluoxetine is an antidepressant of the selective serotonin reuptake inhibitor class. It is manufactured and marketed by Eli Lilly and Company...
(Prozac),
sertralineSertraline hydrochloride is an antidepressant of the selective serotonin reuptake inhibitor class. It was introduced to the market by Pfizer in 1991. Sertraline is primarily used to treat major depression in adult outpatients as well as obsessive–compulsive, panic, and social anxiety disorders in...
(Zoloft),
citalopramCitalopram brand names: Celexa, Cipramil) is an antidepressant drug of the selective serotonin reuptake inhibitor class. It has U.S...
(Celexa),
escitalopramEscitalopram is an antidepressant of the selective serotonin reuptake inhibitor class. It is approved by the U.S...
(Lexapro), and
paroxetineParoxetine is an SSRI antidepressant. Marketing of the drug began in 1992 by the pharmaceutical company SmithKline Beecham, now GlaxoSmithKline...
(Paxil), can be extremely effective in re-regulating the autonomic nervous system and raising blood pressure. Some studies indicate that
serotonin-norepinephrine reuptake inhibitorSerotonin–norepinephrine reuptake inhibitors are a class of antidepressant drugs used in the treatment of major depression and other mood disorders...
s (SNRIs) such as
venlafaxineVenlafaxine is an antidepressant of the serotonin-norepinephrine reuptake inhibitor class. First introduced by Wyeth in 1993, now marketed by Pfizer, it is licensed for the treatment of major depressive disorder , as a treatment for generalized anxiety disorder, and comorbid indications in...
(Effexor) and
duloxetineDuloxetine is a serotonin-norepinephrine reuptake inhibitor manufactured and marketed by Eli Lilly. It is effective for major depressive disorder and has been shown to be as effective as venlafaxine for generalized anxiety disorder...
(Cymbalta) are even more effective. Tricyclic antidepressants, tetracyclic antidepressants, and monoamine oxidase inhibitors are also occasionally, but rarely, prescribed. A combination of two antidepressants, usually an SSRI or SNRI with
bupropionBupropion is an atypical antidepressant and smoking cessation aid. The drug is a non-tricyclic antidepressant and differs from most commonly prescribed antidepressants such as SSRIs, as its primary pharmacological action is thought to be norepinephrine-dopamine reuptake inhibition...
(Wellbutrin) or
mirtazapineMirtazapine is a tetracyclic antidepressant used primarily in the treatment of depression. It is also sometimes used as a hypnotic, antiemetic, and appetite stimulant, and for the treatment of anxiety, among other indications...
(Remeron), is also shown to be very effective.
Medications used to treat attention deficit disorder and attention deficit hyperactivity disorder such as
methylphenidateMethylphenidate is a psychostimulant drug approved for treatment of attention-deficit hyperactivity disorder, postural orthostatic tachycardia syndrome and narcolepsy. It may also be prescribed for off-label use in treatment-resistant cases of lethargy, depression, neural insult and obesity...
(Ritalin) and
AdderallAdderall is a brand name of amphetamine salts–based medication used for attention-deficit hyperactivity disorder and narcolepsy. It is a brand-name psychostimulant medication composed of racemic amphetamine aspartate monohydrate, racemic amphetamine sulfate, dextroamphetamine saccharide, and...
effectively increase norepinehprine and dopamine levels, thereby increasing vasoconstriction and blood pressure.
In the UK
IvabradineIvabradine is a novel medication used for the symptomatic management of stable angina pectoris. It is marketed under the trade name Procoralan , Coralan in India , Australia or such as in Italy Corlentor , and was also known as S-16257 during its development...
has been used to treat patients with POTS symptoms with good effect. Ivabradine acts by reducing the
heart rateHeart rate is the number of heartbeats per unit of time, typically expressed as beats per minute . Heart rate can vary as the body's need to absorb oxygen and excrete carbon dioxide changes, such as during exercise or sleep....
in a mechanism different from
beta blockerBeta blockers or beta-adrenergic blocking agents, beta-adrenergic antagonists, beta-adrenoreceptor antagonists or beta antagonists, are a class of drugs used for various indications. They are particularly for the management of cardiac arrhythmias, cardioprotection after myocardial infarction ,...
s and
calcium channel blockerA calcium channel blocker is a chemical that disrupts the movement of calcium through calcium channels.CCB drugs devised to target neurons are used as antiepileptics. However, the most widespread clinical usage of calcium channel blockers is to decrease blood pressure in patients with...
s, two commonly prescribed
antianginalAn antianginal is any drug used in the treatment of angina pectoris, a symptom of ischaemic heart disease.-Nitrates:Nitrates cause vasodilation of the venous capacitance vessels by stimulating the endothelium-derived relaxing factor...
drugs. It is classified as a
cardiotonic agent.
Anti-anxiety medications, such as
alprazolamAlprazolam is a short-acting anxiolytic of the benzodiazepine class of psychoactive drugs. Alprazolam, like other benzodiazepines, binds to specific sites on the GABAA gamma-amino-butyric acid receptor...
(Xanax),
lorazepamLorazepam is a high-potency short-to-intermediate-acting 3-hydroxy benzodiazepine drug that has all five intrinsic benzodiazepine effects: anxiolytic, amnesic, sedative/hypnotic, anticonvulsant, antiemetic and muscle relaxant...
(Ativan), and
clonazepamClonazepamis a benzodiazepine drug having anxiolytic, anticonvulsant, muscle relaxant, and hypnotic properties. It is marketed by Roche under the trade name Klonopin in the United States and Rivotril in Australia, Brazil, Canada and Europe...
(Klonopin), can be used to combat imbalances of adrenaline usually seen with POTS patients.
- Angiotensin converting enzyme inhibitors, or ACE inhibitors, are used to increase vascular capacity, cardiac output, and sodium and water excretion.
- Clonidine
Clonidine is a sympatholytic medication used to treat medical conditions, such as high blood pressure, some pain conditions, ADHD and anxiety/panic disorder...
can work in patients with reduced sympathetic activity. Clonidine lowers catecholamine(epinephrine and norepinephrine) production.
- Disopyramide
Disopyramide is an antiarrhythmic medication. It is a Class Ia antiarrhythmic used in the treatment of ventricular tachycardias...
(Norpace) is an antiarrhythmic medication that inhibits the release of epinephrineEpinephrine is a hormone and a neurotransmitter. It increases heart rate, constricts blood vessels, dilates air passages and participates in the fight-or-flight response of the sympathetic nervous system. In chemical terms, adrenaline is one of a group of monoamines called the catecholamines...
and norepinephrineNorepinephrine is the US name for noradrenaline , a catecholamine with multiple roles including as a hormone and a neurotransmitter...
.
- Erythropoietin
Erythropoietin, or its alternatives erythropoetin or erthropoyetin or EPO, is a glycoprotein hormone that controls erythropoiesis, or red blood cell production...
, used to treat anemia via intravenous infusion, is very effective at increasing blood volume. It is seldom used, however, due to the dangers of increasing the hematocritThe hematocrit or packed cell volume or erythrocyte volume fraction is the percentage of the concentration of red blood cells in blood. It is normally about 45% for men and 40% for women...
, the inconvenience of intravenous infusion, and its prohibitively expensive cost.
- Pregabalin
Pregabalin is an anticonvulsant drug used for neuropathic pain and as an adjunct therapy for partial seizures with or without secondary generalization in adults. It has also been found effective for generalized anxiety disorder and is approved for this use in the European Union. It was designed...
(Lyrica), an anticonvulsantThe anticonvulsants are a diverse group of pharmaceuticals used in the treatment of epileptic seizures. Anticonvulsants are also increasingly being used in the treatment of bipolar disorder, since many seem to act as mood stabilizers, and in the treatment of neuropathic pain. The goal of an...
drug, has been shown to be especially effective in treating neuropathic painNeuropathic pain results from lesions or diseases affecting the somatosensory system. It may be associated with abnormal sensations called dysesthesia, which occur spontaneously and allodynia that occurs in response to external stimuli. Neuropathic pain may have continuous and/or episodic ...
associated with POTS. In fact, Lyrica was the first and, for its first year on the market the only, prescription drug approved by the FDA to treat fibromyalgiaFibromyalgia is a medical disorder characterized by chronic widespread pain and allodynia, a heightened and painful response to pressure. It is an example of a diagnosis of exclusion...
. Some POTS patients also report improvement in concentration and energy while on Lyrica.
- Pseudoephedrine
Pseudoephedrine is a sympathomimetic drug of the phenethylamine and amphetamine chemical classes. It is used as a nasal/sinus decongestant and stimulant, or as a wakefulness-promoting agent....
and phenylephrinePhenylephrine is a selective α1-adrenergic receptor agonist used primarily as a decongestant, as an agent to dilate the pupil, and to increase blood pressure...
, over the counter decongestants, increase vasoconstrictionVasoconstriction is the narrowing of the blood vessels resulting from contraction of the muscular wall of the vessels, particularly the large arteries, small arterioles and veins. The process is the opposite of vasodilation, the widening of blood vessels. The process is particularly important in...
by promoting the release of norepinephrineNorepinephrine is the US name for noradrenaline , a catecholamine with multiple roles including as a hormone and a neurotransmitter...
.
- Pyridostigmine
Pyridostigmine is a parasympathomimetic and a reversible cholinesterase inhibitor. Since it is a quaternary amine, it is poorly absorbed in the gut and does not cross the blood-brain barrier, except possibly in stressful conditions.-Mode of action:...
(Mestinon), inhibits the breakdown of acetylcholineThe chemical compound acetylcholine is a neurotransmitter in both the peripheral nervous system and central nervous system in many organisms including humans...
, promoting autonomic nervous system activity. It is especially effective in patients who exhibit symptoms of excessive sympathetic activity.
- Theophylline
Theophylline, also known as dimethylxanthine, is a methylxanthine drug used in therapy for respiratory diseases such as COPD and asthma under a variety of brand names. Because of its numerous side-effects, the drug is now rarely administered for clinical use. As a member of the xanthine family, it...
, a drug used to treat respiratory diseases such as COPDChronic obstructive pulmonary disease , also known as chronic obstructive lung disease , chronic obstructive airway disease , chronic airflow limitation and chronic obstructive respiratory disease , is the co-occurrence of chronic bronchitis and emphysema, a pair of commonly co-existing diseases...
and asthmaAsthma is the common chronic inflammatory disease of the airways characterized by variable and recurring symptoms, reversible airflow obstruction, and bronchospasm. Symptoms include wheezing, coughing, chest tightness, and shortness of breath...
, is occasionally prescribed at low doses for POTS patients. Theophylline increases cardiac output, increases blood pressure, and stimulates epinephrineEpinephrine is a hormone and a neurotransmitter. It increases heart rate, constricts blood vessels, dilates air passages and participates in the fight-or-flight response of the sympathetic nervous system. In chemical terms, adrenaline is one of a group of monoamines called the catecholamines...
and norepinephrineNorepinephrine is the US name for noradrenaline , a catecholamine with multiple roles including as a hormone and a neurotransmitter...
production. Due to its very narrow therapeutic indexThe therapeutic index is a comparison of the amount of a therapeutic agent that causes the therapeutic effect to the amount that causes death or toxicity ....
, Theophylline is known to cause a wide variety of side-effects and even toxicityToxicity is the degree to which a substance can damage a living or non-living organisms. Toxicity can refer to the effect on a whole organism, such as an animal, bacterium, or plant, as well as the effect on a substructure of the organism, such as a cell or an organ , such as the liver...
.
- Women who report a worsening of symptoms during menstruation
Menstruation is the shedding of the uterine lining . It occurs on a regular basis in sexually reproductive-age females of certain mammal species. This article focuses on human menstruation.-Overview:...
will often use combined (containing both estrogen and progestin) forms of hormonal contraceptionHormonal contraception refers to birth control methods that act on the endocrine system. Almost all methods are composed of steroid hormones, although in India one selective estrogen receptor modulator is marketed as a contraceptive. The original hormonal method—the combined oral contraceptive...
to prevent hormonal changes and an aggravation of their condition.
External body pressure
Pressure garments can reduce symptoms associated with
orthostatic intoleranceOrthostatic intolerance is a subcategory of dysautonomia, a disorder of the autonomic nervous system occurring when an individual stands up....
by constricting blood pressures with external body pressure.
Compression devices, such as abdominal binders and compression stockings, help to reduce the amount of pooling blood. Compression stockings should be at least 30–40 mm Hg and will work best if they are waist high. Compression stockings should be fitted to achieve the greatest benefit.
Compressions suits (
G-SuitsA G-suit, or the more accurately named anti-G suit, is worn by aviators and astronauts who are subject to high levels of acceleration force . It is designed to prevent a black-out and G-LOC caused by the blood pooling in the lower part of the body when under acceleration, thus depriving the...
) have also been used with some good results.
Changes in environment
Some patients report that symptoms worsen with changes in barometric pressure (for instance, before a thunderstorm) and changes from outdoors to indoors (presumably barometric pressure is higher inside) and depending on weight of clothes and coverage. These patients may find relief by moving to a new location where barometric pressure is relatively stable, e.g. San Diego.
History
POTS was first named and identified by Schondorf and Low in 1993; however, the syndrome has been described in medical studies dating back to at least 1940. Hypertension associated with POTS has been previously described as the "hyperadrenergic syndrome" by Streeten and as "idiopathic hypovolemia" by Fouad. Hypotension associated with POTS has been previously described as the "neurally mediated hypotension" form of POTS.
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