What Is POTS??

What Is POTS??

Wednesday, September 21, 2016

Morbidity--Can You Die From POTS or Associated Conditions?

This post is going to be a hard one for me to write and probably a hard one for some people to read. The possibility or your own mortality is a subject most people try not to think about.

The reason I am writing this is because I had some people get angry at me in a facebook group for suggesting that you could die from POTS.

While it is true that POTS itself doesn't kill people, there is a series of possible events that can ultimately result in death. And I know it to be a fact because my husband died from it. This is what happens:

Hypotension>hypoperfusion>syncope>seizure>cardiac event.
 And it can progress quickly.
My husband went outside, got too hot came in and sat down and a few minutes later, about five, he was in cardiac arrest. And he was never revived by either the EMTs or the ER doctors. Officially, his death certificate says cardiac arrest. But he passed out, had a seizure and then his heart stopped.

I am going to try and explain as well as I can how that is possible.

MASTOCYTOSIS
Here is an article about a man who had a heart attack because he had mastocytosis, which caused him to have syncope, and then the heart attack. Shortness of breath, syncope, and cardiac arrest caused by systemic mastocytosis

Mast cells secrete histamines that cause rashes and hives in an allergic reaction. They also play a role in wound healing and in the immune system.

When someone's mast cells cause them problems, they can have abdominal pain, cramping, diarrhea, flushing, itching, wheezing, coughing, lightheadedness and potential problems with “brain fog” or other difficulties with memory. And there are some patients who have POTS and MCAS/Mast Cell Activation Syndrome. You can read more about POTS and MCAS here: A Tale of Two Syndromes – POTS and MCAS Mastocytosis is when too many of those mast cells remain in the tissues of the body.

 So there is one way POTS can cause a cardiac event, even if it is indirectly because of MCAS.

“Syncope is defined as a sudden loss of consciousness with loss of stature. It is due to the sudden decrease of oxygenated blood perfusion to the brain. If the cause of cerebral hypoperfusion resolves within 1-2 minutes, the consciousness returns. It is not uncommon to observe convulsions during the episode of syncope, which is attributed to the sudden lack of oxygen and glucose to neurons resulting in disorganized neuronal activity. If the cause of cerebral hypoperfusion persists and the individual does not get prompt medical help with cardiopulmonary resuscitation (CPR), permanent damage may occur.” https://www.hawaii.edu/medicine/pediatrics/pedtext/s07c08.html

“It is not uncommon that due to the loss of cerebral perfusion, convulsions can occur for a few seconds or even up to a minute.”

So this says that regardless of what causes the syncope if you don't have prompt medical attention it can progress to the point of needing CPR and permanent damage. Seizures can actually last longer than it says above. “Although usually brief, the convulsions associated with syncope can be quite severe and prolonged.” http://onlinelibrary.wiley.com/doi/10.1111/j.1528-1157.1997.tb01086.x/pdf

Convulsions or seizures in relation to syncope are complicated. Seizure activity in the brain can cause hypotension and syncope. But hypotension and syncope can cause seizures. “While neurally mediated syncope may mimic seizure-like activity, it should also be acknowledged that seizure foci in certain cerebral sites (particularly the temporal lobe) may be the source of apparent neurally mediated syncopal events. Localized seizure activity may initiate the reflex arc previously described, leading to hypotension and bradycardia.” The pathophysiology of common causes of syncope

Bradycardia can happen as a result of a seizure. http://www.seizure-journal.com/article/S1059-1311(14)00070-3/fulltext



"Convulsions suggest prolonged or severe brain hypoperfusion. Shakiness, which can accompany hyperadrenergic activity, can simulate seizure. The duration of loss of consciousness as well as the position of the patient during loss of consciousness is important information. Urinary incontinence and tongue biting during a spell favor a seizure event.

Helping the patient sit or lie down quickly and raising the legs above the heart level permit faster recovery in patients with a typical reflex postural hypotension event. Physicians should check the pulse for amplitude and rhythm. When a patient recovers the acute event, ambulation should be resumed with care because recurrence of hypotension may be inevitable at this stage due to circulatory instability. Oral hydration with salty fluids usually is helpful in the early recovery phase if the patient has no known previous history of heart disease. Serious arrhythmogenic events, coronary insufficiency syndromes, pulmonary embolism, strokes or transient ischemic attacks, and blood loss must be recognized for proper immediate medical care. Injuries sustained during a sudden fall require immediate attention." Cleveland Clinic--Syncope

Asystole is a cardiac arrest rhythm in which there is no discernible electrical activity on the ECG monitor. Asystole is sometimes referred to as a “flat line.”

SUDEP sudden unexpected death in epilepsy.

Apnea is when you stop breathing.

“A principal finding of this study was that apnea and asystole occurred in all SUDEP cases in the early postictal phase after generalized tonic–clonic seizures.” The same article says that usually the asystole or flat-line stops when hypoperfusion and anoxia return to normal. This just means that during a seizure blood flow and oxygen are being interupted but for some people it doesn't return to normal and they die.

“ In conclusion, this patient most probably presented TLOC(total loss of consciousness) episodes due to both reflex syncope and temporal lobe seizures (without a clearly proven link between seizures and syncope).” So you can have both syncope and seizures. The article also says that if seizures are causing the asystole/heart stopping, that a pacemaker is a good treatment option. It was written in 2014. And my husband never lived long enough for us to learn about this. http://www.seizure-journal.com/article/S1059-1311(14)00070-3/fulltext I found a cardiology journal that says the same thing. “Ictal bradycardia/asystole syndrome is mostly related to temporal lobe epilepsy, predominantly in male patients aged older than 50 [44–49]. The diagnosis is based on simultaneous EEG and electrocardiography (ECG) recording with electrographic seizure activity preceding severe bradycardia/asystole or AV-block. A combination of antiepileptic treatment and pacemaker therapy might be required in such patients.” Is it possible to accurately differentiate neurocardiogenic syncope from epilepsy?

“Although convulsive syncope or anoxic seizures are not uncommon, actual epileptic seizures induced by vasovagalor similar forms of syncope have only rarely been reported.” RARE BUT IT DOES HAPPEN. “About 30 s elapsed between the syncope and the onset of the seizure discharge.”
“Of 13 previously reported patients with epileptic seizures induced by syncope, only one had a history of epilepsy with an abnormal interictal EEG.” “it has been speculated by some that a predisposition to generalized epilepsy exists in these patients and that the hypoxic or ischemic syncopal episode might further lower the seizure threshold, resulting in the observed epileptic seizures in these susceptible patients.” http://onlinelibrary.wiley.com/doi/10.1111/j.1528-1157.1997.tb01086.x/pdf

hypotension >Bradycardia>hypoperfusion>sycope>seizure>apnea and asystole/flat-line
seizure>hypotension and bradycardia>hypoperfusion>syncope
That makes two.

Given that one can cause the other, it could be a vicious cycle.


This is another scenario.


This medical paper says that orthostatic hypotension and bradycardia can cause syncope and Atrial fibrillation. “ This is the first report of a patient with persistent atrial fibrillation associated with syncope caused by orthostatic hypotension and bradycardia.” Persistent atrial fibrillation associated with syncope due to orthostatic hypotension: a case report.

This is another article that says that a man had a tilt table test where they induced Afib and that caused him to have syncope. And the next day they did a tilt table test and he had syncope that caused Afib.New England Journal of Medicine--Neurally Mediated Syncope and Atrial Fibrillation


And Atrial Fibrillation can cause sudden cardiac death. “Incident AF is associated with an increased risk of SCD.” Atrial fibrillation and the risk of sudden cardiac death: the atherosclerosis risk in communities study and cardiovascular health study.


hypotension>bradycardia>hypoperfusion>syncope>Afib>Sudden Cardiac Death

And one of the symptoms of POTS is syncope. So, therefore, it follows that the T in POTS or tachycardia causes syncope and therefore can cause Afib and Sudden Cardiac Death.

“The postural tachycardia syndrome (POTS) also presents clinically with symptoms of cerebral hypoperfusion.” Orthostatic Hypertension: When Pressor Reflexes Overcompensate


“This supports the hypothesis that repetitive stable VT(ventricular tachycardia) can play a role in the pathophysiology of cerebrovascular insufficiency.”


“cardiac syncope a variable amount of hemodynamic instability results in cerebral hypoperfusion”

“Systemic hypotension is a common postoperative complication that can cause hypoperfusion and inadequate delivery of oxygen and substrates to organ systems The systemic blood pressure at which the risk of complication increases depends in part on the preoperative blood pressure.”POSTOPERATIVE HYPOTENSION  What this article is getting at is that POTS and orthostatic hypotension patients can go into shock after surgery easier than other people. 

hypotension>hypoperfusion>

Hypoperfusion>syncope
“ These findings suggest that cerebral hypoperfusion, such as with cerebral vasospasms, before the onset of bradycardia might be involved in the mechanism of neurally mediated syncope in patients with an aura.” Possible involvement of cerebral hypoperfusion as trigger of neurally-mediated vasovagal syncope.


syncope>hypoperfusion

hypotension>Afib

“The study suggests that a bout of orthostatic hypotension — a steep blood pressure drop that occurs when a person stands up after a period of lying down — appears to be associated with an overall 40 percent increase in the risk of developing atrial fibrillation over the following two decades.”

Although the above links are referring to orthostatic tachycardia, POTS also is associated with hypoperfusion. Decreased upright cerebral blood flow and cerebral autoregulation in normocapnic postural tachycardia syndrome

"POTS manifests with symptoms of cerebral hypoperfusion and excessive sympathoexcitation.


"The mechanism of POTS is still undergoing a lot of investigation and may be multifactorial. Therefore, any possible overlap with atrial fibrillation is still somewhat unclear and may warrant further study."

Tachycardia>syncope>Afib>Sudden Cardiac Death

It also isn't a far stretch to say:

Tachycardia>syncope>seizure>apnea and asystole/flat-line

Here Is Another Scenario


An enlarged heart isn’t a condition in itself, but a symptom of an underlying problem that is causing the heart to work harder than normal.

“However, if you experience unpleasant symptoms or a permanently increased heart rate is risking heart enlargement, your doctor may recommend treatment with medication or catheter ablation.”Supraventricular Tachycardia

“Tachycardia-induced cardiomyopathy develops slowly and appears reversible by left ventricular ejection fraction improvement, but recurrent tachycardia causes rapid decline in left ventricular function and development of heart failure. Sudden death is possible.” Cardiomyopathy is when the heart muscle becomes enlarged, thick, or rigid. AHA JOURNAL--Heart Failure and Sudden Death in Patients With Tachycardia-Induced Cardiomyopathy and Recurrent Tachycardia

“Some forms of enlarged heart can lead to disruptions in your heart's beating rhythm. Heart rhythms too slow to move blood or too fast to allow the heart to beat properly can result in fainting or, in some cases, cardiac arrest or sudden death.” Mayo Clinic--Complications of Enlarged Heart

So to sum up, tachycardia can cause an enlarged heart which can cause cardiac arrest or sudden death.


A patient can have both seizures and syncope.

Seizures>bradycardia/asystole or AV-block

hypotension >Bradycardia>hypoperfusion>sycope>seizure>apnea and asystole/flat-line SUDEP

seizure>hypotension and bradycardia>hypoperfusion>syncope

orthostatic hypotension and bradycardia can cause syncope and Atrial fibrillation.

hypotension>bradycardia>hypoperfusion>syncope>Afib>Sudden Cardiac Death

Afib>syncope

Tachycardia>syncope>Afib>Sudden Cardiac Death

Tachycardia>hypoperfusion>

Hypoperfusion>bradycaria>syncope

Tachycardia>syncope>seizure>apnea and asystole/flat-line


If those aren't enough for you, here are some more. 


"There are also a number of symptoms and signs that may indicate that a person is at increased risk for SCD. These include:
  • An abnormal heart rate or rhythm (arrhythmia) of unknown cause
  • An unusually rapid heart rate (tachycardia) that comes and goes, even when the person is at rest
  • Episodes of fainting (called syncope) of unknown cause"

RISKS DURING PREGNANCY

There has been debate over whether or not POTS patients should opt for a caesarean section or a vaginal birth.  Due to the physical stress of a vaginal birth, some patients have gone into cardiac arrest (or near cardiac arrest) while delivering.  The reason to opt for a C-section would be to avoid this incidence.  However, a C-section is major surgery and must be discussed thoroughly with a high-risk obstetrician.   Also, some patients may have problems receiving anesthesia which is administered during a C-section and sometimes during vaginal birth (also known as an epidural).  Both of these aspects must be thoroughly reviewed prior to delivery.How does POTS affect pregnancy and labor?



PROBLEMS DURING HEAD UP TILT TABLE TEST
HUT testing is generally safe, but there have been occasional reports of coronary vasospasm, chest pain, hypertensive crisis and tachyarrhythmia. The most frequent adverse effects are hemodynamic changes, such as hypotension, tachycardia or bradycardia associated with orthostatic intolerance, presyncope or syncope. It is noteworthy that patients with neurocardiogenic syncope may rarely experience asystole (defined as ventricular pause of more than 5 seconds) or complete atrioventricular block during HUT testing. Lacroix and colleagues reported 10 asystolic reactions (6%) (average duration 12 seconds) among 179 patients investigated for neurocardiogenic syncope; 8 patients needed cardiopulmonary resuscitation for 20 to 30 seconds. Dhala and associates reported 19 asystolic reactions (9%) among 209 patients with suspected neurocardiogenic syncope and 3 asystolic responses (4%) among 75 healthy control subjects during HUT testing without pharmacologic stimulation. These subjects did not show a worse outcome than their nonasystolic counterparts during follow-up. The fainting patient: value of the head-upright tilt-table test in adult patients with orthostatic intolerance


Autonomic nerve disorders (dysautonomia) refer to disorders of autonomic nervous system (ANS) function. Dysautonomia is a general term used to describe a breakdown or abnormal function of the ANS. 

POTS and Orthostatic hypotension are dysautonomias.

About half of patients with POTS have a restricted autonomic neuropathy with a length-dependent distribution of neuropathy.

"While diabetes is generally the most common cause of autonomic neuropathy, other health conditions — even an infection — may be to blame." http://www.mayoclinic.org/diseases-conditions/autonomic-neuropathy/basics/definition/con-20029053


(This is associated with diabetes but it is possible that other people could have it: see above.)

"Cardiac autonomic neuropathy (CAN) represents a serious complication as it carries an approximately
five-fold risk of mortality in patients with diabetes just as in those with chronic liver diseases. The high
mortality rate may be related to silent myocardial infarction, cardiac arrhythmias, cardiovascular and
cardiorespiratory instability and to other causes not yet explained. Resting tachycardia due to parasympathetic damage may represent one of the earliest signs. Typical findings referring to autonomic dysfunction may include exercise intolerance, orthostatic hypotension
and cardiac dysfunction to rest or exercise. Severe autonomic neuropathy may be responsible for
spontaneous respiratory arrest and unexplained sudden death." Autonomic neuropathy: a marker of cardiovascular risk


"Resting tachycardia due to parasympathetic damage may represent one of the earliest signs of CAN. Experiences from large epidemiological studies indicate that tachycardia of any origin is a major risk factor for cardiovascular and non-cardiovascular death.17 The heart rate-mortality association is observed at any age."

"CAN is associated with a high risk of unexpected and sudden death, possibly related to silent myocardial ischaemia/infarction, cardiac arrhythmias and hypoxia.15 Cardiorespiratory arrests during
or right after anaesthesia have been described."

. Possible factors associated with high mortality and sudden death due to autonomic neuropathy 
● Silent myocardial ischaemia/infarction 
● Cardiorespiratory arrest/increased perioperative and peri-intubation risk 
● Resting tachycardia 
● Ventricular arrhythmias/prolongation of the QT interval 
● Hypertension 
● Orthostatic hypotension 
● Flattening of the nocturnal reduction of blood pressure and heart rate (‘non-dipper’ phenomenon) 
● Exaggerated blood pressure responses with supine position and exercise 
● Abnormal diastolic/systolic left ventricular function 
● Poor exercise tolerance 
● Impaired cardiovascular responsiveness 
● Heat intolerance due to defective sympathetic thermoregulation 
● Susceptibility to foot ulcers and amputations due to arteriovenous shunting and sudomotor dysfunction 
● Hypoglycaemia unawareness (?) 
● Increased risk of severe hypoglycaemia 
● Obstructive sleep apnoea syndrome

Autonomic neuropathy is common in Nigerian patients with non-diabetic Chronic Renal Failure.http://www.ncbi.nlm.nih.gov/pubmed/15171518

Renal Failure isn't something that is associated with POTS but autonomic neuropathy is. 
"The role of obesity is supported by the high prevalence of cardiac autonomic dysfunction in non-diabetic obese people,"Diabetic Cardiovascular Autonomic Neuropathy

"Diabetes can produce the symptoms of POTS (Llamas, Garcia, Gaos, Jimenez, Villavicencio, Cueto & Arriaga, 1985). There are different types of diabetes, including diabetes insipidus, that are associated with POTS symptoms"http://www.dinet.org/index.php/information-resources/pots-place/pots-causes

"POTS is also often classified as primary or secondary.  An example of an 2003 classification of postural tachycardia syndrome by Dr. Grubb (18):

Primary forms: Partial dysautonomic, Immune mediated pathogenesis, Adolescence, Hyperadrenergic state

Secondary forms: Diabetes mellitus, Amyloidosis, Heavy metal poisoning, Sjogren syndrome, Hypermobility syndrome, Paraneoplastic syndrome"

"Both Peripheral (PN), and specifically Small Fiber Neuropathy (known as SFN, a type of PN), have been associated with Autonomic Neuropathy, POTS, and other diseases that cause POTS, such as Diabetes and Sjogrens" what-is-causing-your-pots-and-why-it-is


You can read more on autonomic neuropathy here: Autonomic Neuropathy



Sudden Cardiac Death


Autonomic Neuropathy Causes 2.5 Times a Risk of Sudden Death













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