◆ 12/16/16 Disclosures Postural Orthostatic Tachycardia Syndrome I have nothing to disclose and Inappropriate Sinus Tachycardia Cara Pellegrini, MD Acting Chief, SFVA Associate Professor of Medicine, UCSF MS, a 28 yo F, c/o palps, postural MS, a 28 yo F, c/o palps, postural dizziness, severe fatigue, and inability to dizziness, severe fatigue, and inability to concentrate. concentrate. Supine: HR 73, BP 103/72 Supine: HR 73, BP 103/72 Upright: HR 106, BP 109/80 Upright: HR 106, BP 109/80 Most likely diagnosis: Most likely diagnosis: A. Inappropriate sinus tachycardia A. Inappropriate sinus tachycardia B. Postural orthostatic tachycardia B. Postural orthostatic tachycardia syndrome syndrome C. Autonomic nervous system failure C. Autonomic nervous system failure D. Chronic fatigue syndrome D. Chronic fatigue syndrome ◆ 1
◆ 12/16/16 Overlapping syndromes POSTURAL ORTHOSTATIC TACHYCARDIA SYNDROME POTS Brady PA and colleagues, PACE 2005 Diagnosis Clinical manifestations § HR increase ≥ 30 bpm from supine to § Postural: standing with no orthostatic hypotension § Lightheadedness, (less commonly § Symptoms worsen with standing and syncope,) palpitations, exercise improve with recumbence intolerance § Symptoms persist ≥ 6 months § Unrelated to standing: § Absence of other overt cause of § Nausea, diarrhea, abdominal pain orthostatic symptoms or tachycardia § Systemic: § Fatigue, mental clouding, migraines ◆ 2
◆ 12/16/16 Physiology of upright Epidemiology posture § ~650,000 patients in the US alone Instantaneous descent of ~500mL of blood § Women > men (4:1) 10-25% shift of plasma into interstitial § Usually presents at young age, 15-25yo tissue § Onset often following acute stressor Decreases venous return to heart (preload) § Big impact on QOL § No known mortality § Many have eventual improvement Triggers compensatory sympathetic activation Lower extremity autonomic denervation Hyperadrenergic § Sympathetic postganglionic fibers in LE stimulation Hypovolemia § ↓ sympathetics -> ↓ venoconstriction -> venous pooling - > OI § Anhidrosis of LE § Ganglionic acetylcholine receptor Ab Autonomic POTS denervation Deconditioning ◆ 3
◆ 12/16/16 Blood volumes are Renin-aldosterone paradox decreased Raj SR and colleagues, Circulation 2005 Raj SR and colleagues, Circulation 2005 Increased sympathetic Genetic abnormalities activity § Elevated arterial norepinephrine levels § One kindred with identified mutation in at rest norepinephrine transporter § ↓ norepinephrine clearance § Frequent family history of orthostatic intolerance § Increased resting HR § Larger amplitude sympathetic bursts in peroneal nerve recordings ◆ 4
◆ 12/16/16 The Grinch Syndrome? Exclude other etiologies • Medications • Prolonged bedrest • Dehydration • Anemia • Hyperthyroidism • Pheochromocytoma • Arrhythmia (IST, others) • Cardiomyopathy (postpartum) • Autonomic neuropathies Fu Q and colleagues, JACC 2010 Which HR + BP pattern with Agents that may worsen tilt is most likely POTS? orthostatic intolerance § ACE inhibitors 1. A. 2. B. 3. C. § Alpha receptor blockers § Calcium channel blockers, Beta blockers § Phenothiazines § Tricyclic antidepressants, MAO inhibitors § Bromocriptine § Ethanol, Opiates § Diuretics, Hydralazine, Nitrates, Sildenafil § Ganglionic-blocking agents ◆ 5
◆ 12/16/16 Which HR + BP pattern with Evaluation tilt is most likely POTS? § H+P, including orthostatic vital signs (I) C.* 1. A. 2. B. 3. § ECG (I) § CBC and TFTs (IIa) § 24 hour Holter monitor (IIb) § Autonomic testing (IIb) § Echocardiogram (IIb) § Tilt-table testing (IIb) § Exercise stress testing (IIb) Sheldon RS and colleagues, Heart Rhythm 2015 Nonpharmacologic Dependent Acrocyanosis Treatment § Reverse any contributing condition § Avoid aggravating factors (heat) § Elastic support hose (waist high) § Exercise (recumbent) (IIa) § IVF (emergency therapy) (IIa) § Salt (10-12 g / day) (IIb) § Hydration (8-10 cups of water daily) (IIb) Raj, Indian Pacing and Electrophysiology Journal 2006 Sheldon RS and colleagues, Heart Rhythm 2015 ◆ 6
◆ 12/16/16 Beneficial effects of Which drug is NOT exercise recommended in POTS? Propranolol A. Ritalin B. Methyldopa C. Pyridostigmine D. George SA and colleagues, Heart Rhythm 2016 Which drug is NOT Pharmacologic therapy recommended in POTS? (off label, mostly IIb) § Fludrocortisone (aldosterone analog) Propranolol § Midodrine (vasoconstrictor) A. Ritalin § DDAVP (for special events) B. Methyldopa § Low-dose propranolol, ivrabradine C. Pyridostigmine § Pyridostigmine (acetylcholinesterase D. inhibitor) § Clonidine, methyldopa (hyperadrenergic form) § Modafinil (stimulant) Sheldon RS and colleagues, Heart Rhythm, 2015 § ◆ 7
◆ 12/16/16 Pharmacologic therapy Propranolol – go low! (off label, mostly IIb) Low dose / placebo § Fludrocortisone (aldosterone analog) § Midodrine (vasoconstrictor) § DDAVP (for special events) § Low-dose propranolol, ivrabradine § Pyridostigmine (acetylcholinesterase Low / High dose inhibitor) § Clonidine, methyldopa (hyperadrenergic form) § Modafinil (stimulant) Sheldon RS and colleagues, Heart Rhythm, 2015 § Raj SR and colleagues, Circulation, 2009 Acetylcolinesterase Pharmacologic therapy inhibition (off label, mostly IIb) § Fludrocortisone (aldosterone analog) § Midodrine (vasoconstrictor) § DDAVP (for special events) § Low-dose propranolol, ivrabradine § Pyridostigmine (acetylcholinesterase inhibitor) § Clonidine, methyldopa (hyperadrenergic form) § Modafinil (stimulant) Sheldon RS and colleagues, Heart Rhythm, 2015 Raj SR and colleagues, Circulation 2005 § ◆ 8
◆ 12/16/16 POTS - Summary § Orthostatic symptoms and tachycardia § Lightheadedness, exercise intolerance, INAPPROPRIATE SINUS palpitations, occasionally syncope § Incapacitating but not malignant TACHYCARDIA § Etiology multifactorial: peripheral autonomic denervation, hypovolemia, hyperadrenergic, deconditioning § Exclude reversible causes (meds) § Tx: reconditioning, hydration, salt § Rx: volume expansion, vasoconstriction, HR control, neuromodulators Which Holter is from a Diagnosis patient with IST? § Elevated resting HR B. § Daytime > 100 bpm A. § Mean 24 hour HR >90 bpm § Exaggerated response to exertion § P wave consistent with sinus mechanism § No physiologic basis for tachycardia C. Castellanos A and colleagues, American Journal of Cardiology 1998 Brady PA and colleagues, PACE 2005 ◆ 9
◆ 12/16/16 Which Holter is from a Clinical manifestations patient with IST? B. § Asymptomatic -> incapacitating sx’s A. § Palpitations § Lightheadedness, syncope § Fatigue § Exercise intolerance § Chest pain, shortness of breath All! C. Brady PA and colleagues, PACE 2005 Epidemiology § Primarily young women § Often hypertensive § Healthcare professionals? ? § 1.2% of (middle- aged) population § Often hypertensive § No gender difference Images: Levy MN, Circulation Research 1971 Yusuf S and Camm AJ, Journal of CV Pharmacology and Therapeutics 2003 Still AM and colleagues, Europace 2005 ◆ 10
◆ 12/16/16 In favor of localized autonomic In favor of primary sinus node dysfunction abnormality § High intrinsic heart rate (125% predicted) β -adrenergic § hypersensitivity Impaired vagal § response Normal § sympathovagal balance Bauernfeind RA and colleagues, Annals of Internal Medicine 1979 Morillo CA and colleagues, Circulation 1994 In favor of β -receptor Natural history stimulation by auto-antibodies § Generally benign § Aggravation of symptoms rare Yet § Concern persists for effect on mortality Still AM and colleagues, Europace 2005 Chiale PA and colleagues, Heart Rhythm 2006 ◆ 11
◆ 12/16/16 Exclude secondary causes Evaluation § Anemia § Complete History and Physical (I) § Infection / Fever § ECG (I) § Diabetic neuropathy § CBC and TFTs (IIa) § Hyperthyroidism § 24 hour Holter monitor (IIb) § Cushing’s disease § Exercise stress testing (IIb) § Pheochromocytoma § Urine/serum drug screening (IIb) § Carcinoid § Autonomic testing (IIb) § Structural heart disease Sheldon RS and colleagues, Heart Rhythm 2015 What is the primary goal What is the primary goal of IST treatment? of IST treatment? A. Heart rate reduction A. Heart rate reduction B. Decrease in mortality B. Decrease in mortality C. Improvement in palpitations C. Improvement in palpitations D. Reduction in heart failure risk D. Reduction in heart failure risk ◆ 12
◆ 12/16/16 Conventional Treatment Conventional Treatment § Engagement of the patient § Engagement of the patient § Multidisciplinary team § Multidisciplinary team § Regular exercise § Regular exercise § Avoidance of stimulants § Avoidance of stimulants § Good sleep hygiene § Good sleep hygiene § Beta-blockers § Beta-blockers § Verapamil § Verapamil Ivabradine Mean HR Max HR Min HR Yusuf S and Camm AJ, Journal of CV Pharmacology and Therapeutics 2003 Calo L and colleagues, Heart Rhythm 2010 ◆ 13
◆ 12/16/16 Ivabradine RCT Mean HR Rest HR Max HR Max HR Min HR Cappato R and colleagues, JACC 2012 Calo L and colleagues, Heart Rhythm 2010 Treatment § Seek and treat reversible causes (I) § Ivabradine (IIa) § Sinus node modification (III) § Surgical ablation (III) § Sympathetic denervation (III) Sheldon RS and colleagues, Heart Rhythm 2005 Ptaszynski P and colleagues, Europace 2013 ◆ 14
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