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Postural orthostatic tachycardia syndrome

POTS

Descriere
Este o condiie a disautonomiei, mai specific, o intoleran ortostatic, n care trecerea de la clino la ortostatism produce o tahicardie anormal; de cele mai multe ori se acompaniaz cu o scdere a TA. Debitul sanguin cerebral scade. Pacienii cu POTS au probleme n a-i menine homeostazia fluidelor la schimbarea poziiei, ex. deplasarea de pe un scaun pe altul sau atingerea unor obiecte aflate la nlime, deasupra capului. Muli dintre ei sunt simptomatici n repaus.

Simptomatologie
Simptomatologia difer ca severitate de la un pacient la altul. POTS este o condiie medical serioas, dei ne-amenintoare de via, care poate debilita sever. Unii pacieni nu pot urma cursurile colii, nu se pot desfura profesional, reprezentnd chiar un mare handicap. Hipotensiunea arterial asociat POTS s-a mai denumit "neurally mediated hypotension.

Simptomatologie 2
Esena POTS este o cretere a AV la trecerea din clino n ortostatism cu mai mult de 30 bti pe minut sau la o frecven mai mare de 120 bti pe minut n primele 10 minutes de la headup tilt. Aceast tahicardie este deseori acompaniat de o hipotensiune arterial simptomatic:

ameeli, uneori pre-sincope (pre-lipotimie) (nu vertij) Intoleran la efort Fatigabilitate extrem Sincope.

Hipoperfuzia tisular produce:

Extremiti reci Dureri toracice i disconfort toracic dezorientare dispnee cefalee Slbiciune muscular tremurturi Tulburri vizuale

Disfuncia sistemului autonom poate cauza simptome gastrointestinale:


Dureri abdominale sau discomfort balonri constipaie diaree greuri vrsturi

Hipoperfuzia cerebral produce dificulti cognitive i emoionale:


Cea pe creier epuizare scderea forei mentale depresie dificulti n gsirea cuvntului corect Capacitate redus de concentraie Tulburri de somn

Niveluri nepotrivite de adrenalin i noradrenalin pot produce simptome anxiety-like:


frisoane anxietate flushing hipertermie Nervozitate Simptomele de POTS se pot suprapune peste cele ale tulburrilor anxioase, cu care se poate confunda.

Associated conditions
POTS is often accompanied by vasovagal syncope, 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 disfunction 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 hypoperfusion.

POTS may be a cause of chronic fatigue syndrome in patients that exhibit signs of orthostatic intolerance. Treating POTS will greatly improve or even eliminate disabling fatigue for these patients. Some patients with fibromyalgia 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 in many patients as well. Patients with Ehlers-Danlos syndrome, often develop POTS as a secondary condition. Some POTS patients experience symptoms associated with Restless Leg Syndrome, or RLS. Treating POTS should also relieve RLS symptoms in these patients.

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 mononucleosis or pneumonia. 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 pregnancy. 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 dysautonomia 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 intolerance. Some patients have been found to have a significant increase in upright dopamine 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 low plasma volume (hypovolemia), 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 underactivty can also occur in some forms of orthostatic intolerance, such as pure autonomic failure.

Diagnosis
POTS can be difficult to diagnose. A routine physical examination and standard blood tests will not indicate POTS. A tilt table test is vital to diagnosing POTS, although all symptoms must be considered before a final diagnosis is made. Tests to rule out Addison's Disease, pheochromocytoma, electrolyte imbalance, Lyme Disease, Celiac Disease, and various food allergies are usually performed. A blood test may be performed to verify abnormally high levels of norepinephrine 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 pregnancy.

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 overtime and have lifelong symptoms. Patients with secondary POTS as a consequence of Ehlers-Danlos Syndrome will also usually struggle with symptoms for life. 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.

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 has been shown to drastically exacerbate all types of orthostatic intolerance due to its vasodilation and dehydration properties. In addition to its adverse effects, it interacts unfavorably with many of the medications prescribed to 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, 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 hypotension by helping the body retain water and thereby expand 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 helps some POTS patients due to its stimulative effects; however, other patients report a worsening of symptoms with caffeine intake.

Physical therapy and exercise


POTS symptoms can be worsened by postural asymmetries, restrictions in mobility, and areas of adverse mechanical tension in the nervous system. These physical abnormalities can be relieved with gentle manual therapies including neural mobilization (or neural tension work), myofascial release, and cranio-sacral therapy. Exercise is very important for maintaining muscle strength and avoiding deconditioning. Though many POTS patients report difficulty exercising, some form of exercise is essential to controlling symptoms and eventually, improving the condition. Aerobic exercise performed for 20 minutes a day, three times a week, is sometimes recommended for patients who can tolerate it. Wearing ankle weights compels the leg muscles to work harder, thereby forcing pooling blood back into the upper body. It is especially helpful to put the weights on before getting out of bed in the morning. Ankle weights should not be worn continuously, as the body will then adjust to the extra weight and they will no longer be effective. Ankle weights should be used with caution, as they can possibly interfere with one's normal gait which can cause injury.

Medications
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 fludrocortisone, or Florinef, a corticosteroid 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. Gatorade is also effective in providing both sodium and fluid

Beta blockers such as atenolol and propanolol are often prescribed to treat POTS. These medications work by blocking the effects of epinephrine and norepinephrine released by the autonomic nervous system. Beta blockers also reduce sympathetic activity by blocking sympathetic impulses. For some patients, beta blockers increase POTS symptoms.

Midodrine (Proamatine), is approved by the U.S. FDA to treat orthostatic hypotension, a condition related to POTS. It is a stimulant that causes vasoconstriction and thereby increases blood pressure 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). Antidepressants, especially selective serotonin reuptake inhibitors (SSRIs) such as Prozac, Zoloft, Celexa, Lexapro, and Paxil, can be extremely effective in re-regulating the autonomic nervous system and raising blood pressure. Some studies indicate that serotonin-norepinephrine reuptake inhibitors (SNRIs) such as Effexor and 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 Wellbutrin or Remeron, is also shown to be very effective.

Medications used to treat ADD and ADHD such as Ritalin and Adderall are used to balance dopamine levels, increase vasoconstriction, and increase blood pressure. In the UK Ivabradine has been used to treat patients with POTS symptoms with good effect. Ivabradine acts by reducing the heart rate in a mechanism different from beta blockers and calcium channel blockers, two commonly prescribed antianginal drugs. It is classified as a cardiotonic agent. Anti-anxiety medications, such as Xanax, Ativan, and 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 vasoconstriction, cardiac output, and sodium and water retention. Clonidine can work in patients with reduced sympathetic activity. Ironically an anti-hypertensive drug, Clonidine promotes production and release of epinephrine and norepinephrine. Disopyramide, or Norpace, is an antiarrhythmic medication that inhibits the release of epinephrine and norepinephrine. Erythropoietin, 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 hematocrit, the inconvenience of intravenous infusion, and its prohibitively expensive cost. Pregabalin, or Lyrica, an anticonvulsant drug, has been shown to be especially effective in treating neuropathic pain associated with POTS. In fact, Lyrica is currently the only prescription drug approved by the FDA to treat fibromyalgia. Some POTS patients also report improvement in concentration and energy while on Lyrica.

Pseudoephedrine and phenylephrine, over the counter decongestants, increase vasoconstriction by promoting the release of norepinephrine. Pyridostigmine, or Mestinon, inhibits the breakdown of acetylcholine, promoting autonomic nervous system activity. It is especially effective in patients who exhibit symptoms of excessive sympathetic activity. Theophylline, a drug used to treat respiratory diseases such as COPD and asthma, is occasionally prescribed at low doses for POTS patients. Theophylline increases cardiac output, increases blood pressure, and stimulates epinephrine and norepinephrine production. Due to its very narrow therapeutic index, Theophylline is known to cause a wide variety of side-effects and even toxicity. Women who report a worsening of symptoms during menstruation will often use combined (containing both estrogen and progestin) forms of hormonal contraception to prevent hormonal changes and an aggravation of their condition.

External body pressure


Pressure garments can reduce symptoms associated with orthostatic intolerance 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-Suits) have also been used with some good results

Inappropriate sinus tachycardia (IST)

Definitie
Inappropriate sinus tachycardia (IST) is an uncommon type of cardiac arrhythmia, within the category of supraventricular tachycardia(SVT). The mechanism and primary etiology of Inappropriate sinus tachycardia has not been fully elucidated. The mechanism of the arrhythmia primarily involves the Sinus Node and perinodal tissue and does not require the AV Node (Atrioventricular node) for maintenance. Treatments in the form of pharmacological therapy or Catheter ablation are available, although it is currently difficult to treat successfully.

Diagnostic
Exclusion of all other causes of Sinus tachycardia Common forms of Supraventricular tachycardia (SVT) must be excluded Normal P wave morphology A resting Sinus tachycardia is usually (but not always) present Nocturnal dip in Heart rate Inappropriate Heart rate response on exertion Mean Heart rate in 24hrs >95bpm Symptoms are documented to be due to tachycardia Hypotension is occasionally observed Syncope (fainting) is occasionally reported

Tratament
IST has been treated both pharmacologically and invasively, with varying degrees of success. Some types of medication tried by cardiologists and other physicians include: Beta blockers, Calcium channel blockers and Antiarrhythmic agents. Some SSRI drugs are also occasionally tried and also treatments more commonly used to treat Postural orthostatic tachycardia syndrome such as Fludrocortisone. This approach is very much "trial-and-error". Patients with IST are often intolerant to Beta blockers. A new selective sinus node inhibitor Ivabradine is also being used to treat IST

Invasive treatments include


forms of Catheter ablation such as Sinus Node Modification (selective ablation of the Sinus Node), Complete Sinus Node Ablation (with associated implantation of a Permanent Artificial pacemaker) and AV Node Ablation in very resistant cases (creation of iatrogenic complete heart block, necessitating implantation of a Permanent Artificial pacemaker).