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        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”    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  Patients with both POTS and NCS tend to pool more blood into their veins.    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. (%)               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  Thus POTS is a heterogenous disorder Developmental  Autoimmune  Genetic  Chronic Hypovolemia  Secondary Causes  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  Secondary to other causes. A state of peripheral autonomic deinnervation or vascular unresponsiveness         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         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      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          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 1. 2. 3. 4. 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 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. Fludrocortisone / DDAVP Methylphenidate Midodrine Beta blockers SSRIs Clonidine Erythropoietin Yohimbine Pyridostigmine Norepinephrine reuptake inhibitors Octreotide LDOPS Modafinil Medications   Increase blood volume  Fludrocortisone  DDAVP (desmopressin acetate)  Clonidine  Oral contraceptive pills Improve venous constriction  Midodrine  Methylphenidate  Modafinil  Erythropoeitin Medications  Block response to catecholamines (lower heart rate)  Beta blockers (propranolol, nadalol, atenolol)  Assist neurotransmitters (serotonin, norepinephrine) in the CNS to regulate the autonomic nervous system  Antidepressants (citalopram, fluoxetine, venlafaxine, bupropion, duloxetine) Medications    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