Download Syncope and Postural Tachycardia Syndrome:

Syncope and Postural Tachycardia Syndrome:
Understanding why people faint and what can be done about it.
Beverly Karabin PhD CNP
Associate Professor School of Nursing
University of Toledo
Nurse Practitioner Autonomic Disorders/Syncope Clinic
University of Toledo Medical Center
Toledo, Ohio USA
Special thanks to Blair P Grubb MD.
Medtronic
Biotronik
Orthostatic Intolerance:
Is an umbrella term for several conditions of
abnormal autonomic control
which are made worse upon standing that are
generally relieved when supine.
Orthostatic intolerance
Symptoms include exercise intolerance, fatigue,
lightheadedness, diminished concentration,
sweating disorders, tremulousness, nausea, GI
disturbances, nocturia, headache, neck pain, near
syncope, and syncope
Joint Consensus Statement of the American Autonomic Society and
the American Academy of Neurology; Low, PA et al. Mayo Clinic Proce: 1995; 70:617-22
Syndromes associated with
chronic orthostatic intolerance
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Postural Orthostatic Tachycardia Syndrome (POTS)
Neurocardiogenic Syncope (NCS) {Vasodepressor
Syncope, Neurally Mediated Hypotension (NMH)}
Autonomic Failure Syndromes
The syndromes may overlap
Treatment is similar
Mechanisms are different
All may lead to syncope or transient loss of
consciousness (TLOC) {fainting, blackouts}
Disorders of the Autonomic Nervous System
Reflex/NCS
Syncope
Pure
Autonomic
Failure
Postural
Orthostatic
Tachycardia
Syndrome
Multiple
System
Atrophy
Postural Tachycardia Syndrome (POTS)
Characterized by sinus tachycardia (or an
exaggerated heart rate) and other symptoms of
orthostatic intolerance provoked by upright
position and relieved by recumbence in which
symptoms appear to be due to the reduction of
cerebral blood flow
Brady, Low & Shen (2005). PACE (28) 1112-1121
A healthy person has a slight increase in heart
rate upon standing usually 10-15 beats within
the first 10 minutes of standing.
POTS is an abnormal heart rate response to
upright posture
POTS Criteria
POTS is characterized by an increase in the heart rate at
least 30 beats per minute in the first 10 minutes of
standing or on head upright tilt table test
Or if the heart rate reaches 120 beats per minute in the
first 10 minutes of standing or head upright tilt table
test
In addition the individual has longstanding (>6 months)
and disabling orthostatic intolerance symptoms
The individual has an absence of an underlying cause
(debilitating disease, dehydration, medications,
etc…)that may result in upright tachycardia
In POTS the heart itself is usually NORMAL
In POTS the syndrome does not lead to heart disease
The syndrome of POTS does not lead to a shortened life span
POTS patients hearts don’t “wear out”
POTS may be functionally
debilitating
Maintenance of Postural Blood Pressure
Regulated by a complex process that involves
the skeletal muscles, venous valves, the
autonomic nervous system (short term)
and renin-angiotension system (long term)
Carlson & Grubb (2011). Diagnosis and management of syncope. Pp 1125-1138.
In Fuster, Walsh, Harrington (Eds.) Hurst’s The Heart (13th edition). McGrawHall. NY, NY.
Effects of upright posture
Nature gives you a backup system called the skeletal
muscle pump, or the contraction of leg, abdomen
and arm muscles that compress the venous system
to help propel blood back to the heart.
“a healthy good set of legs can raise the blood
pressure by up to 10-15 points”
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For most of us the skeletal muscle pump is back
up system.
It is not designed to be a full time operating
system and at times it may fail, which may result
in increased venous pooling.
In people with poor autonomic tone they
unconsciously become very dependent on this
system.
Upright posture with POTS and NCS
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Patients with both POTS and NCS tend to pool more
blood into their veins.
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POTS: excessive pooling causes increase in heart rate
NCS: pooling causes a reflex decrease in heart rate and more
vein pooling (leading to low bp and syncope)
Both syndromes have similar symptoms because they
result in less blood flow to the brain.
Once upright you must maintain cerebral blood flow
otherwise gravity will displace blood downward
Tired, fatigue
Poor focus, diminished concentration, brain fog,
cognitive impairment
Lightheaded, dizziness, vertigo
Vision disturbances- black spots, tunnel vision, grey out
Tremulousness, tingling, TIA like symptoms
Near Syncope
Syncope
Convulsive Syncope
70 b/m
100/70 mm/hg
Patients with classic neurocardiogenic
syncope have intermittent symptoms and
episodes of syncope that are generally separated
by long periods of time.
Patients with POTS have near constant
symptoms of orthostatic intolerance.
As in heart failure, it is as if
patients are “running in place” all the time.
However both syndromes may look similar.
Da Costa JM: On Irritable heart: A clinical study of a
Functional cardiac disorder and it’s consequences.
Am J Med Sci 1871:61:17-52
“Dizziness,headache, chest pain, faintness and
extreme fatigue associated with a rapid heart rate upon
standing that fell to normal levels with recumbency”
Case # 12 : 122 beats/min standing- 90 bpm supine
“in all, the immediate effect of the
Exchange in position was most striking”
Venous Pooling in POTS: acral cyanosis
Supine
Normal
Upright
Pooling
Symptoms in POTS Pts. (%)
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Lightheadedness
Dizziness
Palpitations
Exercise Intolerance
Blurred Vision
Chest discomfort
Clamminess
Near Syncope
Anxiety
Flushing
Syncope
Fatigue
Headache
Dyspnea
85-95
60-80
40-55
50-85
70
60
60
50
50
50
40-45
45-75
50
40
Grubb & Olshansky, 2005
Figure I: Subtypes of Postural Tachyca rdia Synd rome
POTS
primary
hyperadrenergic
partial
dysautonomic
developmental
post
viral
secondary
JHS
diabetes
paraneop lastic
other
other
POT S = Postural Tachycardia Syndrome
JHS = Joint Hypermobility Syndrome
There are many different mechanisms that
may result in POTS
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Thus POTS is a heterogenous disorder
Developmental
 Autoimmune
 Genetic
 Chronic Hypovolemia
 Secondary Causes
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Developmental POTS
1.
2.
3.
4.
5.
Unique to adolescents, mainly young women
Onset around 14 yrs
Usually follows a period of rapid growth
Peak symptoms usually around 16 yrs
Tend to be similar to the PD form
a. Orthostatic Intolerance/Tachycardia/Syncope
b. GI problems (nausea,constipation)
c. Exercise intolerance
d. Cognitive impairment
e. Blurred vision
f. Acral cyanosis
g. Severe fatigue
h. Severe frequent migraines
6. Slow steady improvement over 3 - 5 yrs in 70-80 %
Secondary Causes of POTS
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Secondary to other causes. A state of peripheral
autonomic deinnervation or vascular unresponsiveness
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Diabetic
Connective Tissue Disease
Amyloidosis, Sarcoidosis
Chemotherapy medications
Heavy metal poisoning
Alcoholism
Paraneoplastic syndromes
Joint hypermobility syndrome (JHM)
(Grubb, BP. 2008. Circulation (117) 2814-2817.)
Primary POTS: hyperadrenergic
The Vanderbilt group has isolated a
gene defect in a hereditary form of
POTS affecting a norepinephrine
transporter substance.
NEJM 2000
Primary Postural Tachycardia:
Hyperadrenergic
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Upright hypertension (high blood pressure)
High serum upright norepinephrine > 600pg/ml
Tremor
Panic attacks, anxiety
Severe migraines
Diarrhea
Hyperhydrosis (excessive sweating) clamminess
Excessive isoproterenol response to tilt
POTS Onset
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Infections (post viral)
Postpartum
Traumatic
Surgery
Sepsis
Thieben et al 2007. Mayo Clinic Proceedings. (82) 308-313
Other reported cases of POTS
Lightning Injury
Electrical Injury
Pregnancy
Postpartum
Multiple Sclerosis
Traumatic Brain Injury
Lyme Disease
(Kanjwal, Karabin, Kanjwal, Grubb, 2007, 2009, 2010, 2011)
Post Ablation AVNRT
Concurrent with Neurocardiogenic Syncope
(Kanjwal, Sheikh, Karabin, Kanjwal, Grubb 2010, 2011)
Mitochondrial Cytopathy
(Kanjwal Karabin, Kanjwal, Saeed, Grubb 2010)
TREATMENT OPTIONS
Fixing Ourselves
Avoid /Minimize Triggers
Prolonged standing
 Prolonged sitting
 Hot showers, saunas
 Hot crowed rooms
 Hot weather
 During or after exercise
 In the morning
 Changes in barometric
pressure, altitude, travel
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Strong emotions
Anxiety
Medical procedures
Dehydration-illness
After eating
Gory scenes
Alcohol, medications
Abrupt changes in
hormones
Remember you can “do everything right”
and still have symptoms
This is discouraging!!
Before embarking on medical
therapy one must:
1.
2.
3.
Avoid predisposing conditions or medications
Have adequate fluid (2-3 liters/day) & salt
intake (3-5 gram/day: except hyperadrenergic)
Reconditioning and lower extremity strength
building
a. aerobic training 30 min. 3/week
b. resistance training
c. physical therapy, aquatic therapy, cardiac
rehabilitation or personal training
Physical reconditioning
Calm senses
Rejuvenate
Relax
Non-Pharmacotherapy
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Diet, consider food hypersensitivities (gluten,
lactose)
Counter pressure maneuvers (arms, legs,
abdomen)
Support Hose (waist high 30 mmhg ankle
compression), abdominal binders
Elevate head of bed (10-15 degrees)
It is sometimes necessary to begin
medications to
make the patient feel well enough
so that they can begin a reconditioning
program
Pharmacotherapy Pearls
Consider the type
Primary partial dysautonomic (PD) POTS
Hyperadrenergic POTS
Consider co-morbidities
Depression, migraines, obesity, pregnancy,
hypertension
Consider age
Adolescence, adulthood
Consider symptom control
What symptom is the most troublesome? (headaches,
exercise intolerance, tachycardia, neuropathy, fatigue,
dizziness, nausea?)
Pharmacotherapy
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Fludrocortisone / DDAVP
Methylphenidate
Midodrine
Beta blockers
SSRIs
Clonidine
Erythropoietin
Yohimbine
Pyridostigmine
Norepinephrine reuptake inhibitors
Octreotide
LDOPS
Modafinil
Medications
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Increase blood volume
 Fludrocortisone
 DDAVP (desmopressin acetate)
 Clonidine
 Oral contraceptive pills
Improve venous constriction
 Midodrine
 Methylphenidate
 Modafinil
 Erythropoeitin
Medications
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Block response to catecholamines (lower heart rate)
 Beta blockers (propranolol, nadalol, atenolol)
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Assist neurotransmitters (serotonin, norepinephrine) in
the CNS to regulate the autonomic nervous system
 Antidepressants (citalopram, fluoxetine, venlafaxine,
bupropion, duloxetine)
Medications
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Improve constriction of mesentaric (gut) vessels
 Somatostatin (Octreotide)
Central nervous system regulation of (GABA, others)
neurotransmitters: use in migraines/neuropathy
 Topipramate (Topamax)
 Gabapentin (Neurontin)
 Pregabalin (Lyrica)
Assist peripheral nervous system transmission of
acetylcholine, a neurotransmitter
 Pyridostigmine (Mestinon)
POTS patients suffer a degree of functional
impairment similar to that of patients
with COPD or CHF
Benrud-Larson et al, Quality of life in patients with postural
tachycardia syndrome. Mayo Clinic Proceedings 2002: 77, 531-537
Psychosocial aspect of POTS
Pots can be a functionally debilitating illness
The illness may alter relationships, family dynamics,
employment, academics, and overall quality of life
Our goal should be to assist the patient with all
aspects of functioning, including physical,
emotional, spiritual and mental well being.
Remember that individuals with a chronic illness are normal
children, teenagers, partners, friends, colleagues
Blair P Grubb MD
Professor of Medicine
and Pediatrics
Director Autonomic
Disorders Clinic
University of Toledo
Medical Center
USA
Champion of
Dysautonomia
Man never made any material as
resilient as the human spirit
Bern William