docAutonomic & Neurovascular Medicine Unit, Faculty of Medicine, Imperial College London
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Autonomic & Neurovascular Medicine Unit, Faculty of Medicine, Imperial College London
Autonomic & Neurovascular Medicine Unit, Faculty of Medicine, Imperial College London at St Mary’s Hospital. Autonomic Unit, National Hospital for Neurology & Neurosurgery, Queen Square & Institute of Neurology, University College London The Autonomic Clinic - Why is it so special? Dr Valeria Iodice, MD Miss Catherine Best, RN Dr David Low, PhD The Autonomic Clinic • 1. Postural Tachycardia Syndrome and Syncope – Dr Valeria Iodice, Clinical Research Fellow • 2. Treatment Strategies – Miss Catherine Best, Nurse Specialist • 3. Clinical Research Aspects – Dr David Low, Clinical Research Associate Fellow Postural Tachycardia Syndrome Aims of treatment • Awareness and education • Prevent collapse and near collapse • Reduce the frequency and intensity of symptoms • Improve quality of life Postural Tachycardia Syndrome (PoTS) and Autonomic (Neurally) Mediated Syncope (AMS) Dr Valeria Iodice, MD Autonomic Neurovascular Clinical Team Prof. C.J. Mathias Clinical Director Dr G. Ingle Consultant Neurologist Clinicians SHO/Registrar Clinical Fellows 1 Autonomic Nurse Specialist 11 Clinical Autonomic Scientists 4 Clinical Secretaries Overview • PoTS and AMS definition • Diagnosis/role of autonomic evaluation • Associations/allied features Postural Tachycardia Syndrome (PoTS) • • • • • • • More females than males 25-40 years Postural and exertional palpitations Lightheadedness, fatigue, tremulousness Syncope Disproportionately disabled Symptoms with heart rate rise of >30 beats/min or >120 beats/min without orthostatic hypotension 60 O Head up tilt 200 140 Normal Heart Rate bpm Blood Pressure mmHg (Portapres) 10 MIN OF 60°HEAD UP TILT 0 0 200 140 PoTS 10 MIN OF 60°HEAD UP TILT 0 0 Autonomic (Neurally) Mediated Syncope • Transient loss of consciousness in response to a trigger 120 120 60°HEAD UP TILT 0 0 Triggers • Orthostatic stress • Blood phobia • Exercise • Food • Hot environment PoTS and AMS, what in common? • Intermittent episodes of autonomic dysfunction OH PoTS AMS Normotension Bradycardia/hypotension Orthostatic Tachycardia Orthostatic Hypotension Intermittent autonomic dysfunction Fixed autonomic dysfunction Diagnosis • Clinical history • Autonomic evaluation - Autonomic screening tests - Additional autonomic tests 60 O Head up tilt 60 O Head up tilt 200 140 Normal Heart Rate bpm Blood Pressure mmHg (Portapres) 10 MIN OF 60°HEAD UP TILT 0 0 200 140 PoTS 10 MIN OF 60°HEAD UP TILT 0 0 60 O Head up tilt 200 140 Normal Heart Rate bpm Blood Pressure mmHg (Portapres) 10 MIN OF 60°HEAD UP TILT 0 0 Vasovagal syncope VENEPUNCTURE 120 120 140 60°HEAD UP TILT 0 160 60°HEAD UP TILT 0 0 0 Pressor Response 250 Cutaneous Cold 100 Normal Heart Rate bpm Blood Pressure mmHg (Portapres) 0 90 SECONDS Isometric Exercise 140 SECONDS OF RIGHT ARM ISOMETRIC EXERCISE 0 Valsalva Manoeuvre 160 80 Normal Heart Rate bpm Blood Pressure mmHg (Portapres) 0 EXPIRATION 15 SECONDS Deep Breathing 10 SECONDS OF DEEP BREATHING 0 Do we need additional autonomic tests? Modified Exercise testing 140 120 129 100 65 72 pre ex stand 40 96 supine rest Heart rate80 bpm 60 20 post ex stand supine rest 0 Liquid Meal challenge Heart rate bpm Blood pressure (mmHg) 60 O Head up tilt Pre - meal Post - meal 24 hour BP & HR profile in PoTS 180 180 STANDING STANDING AFTER EXERCISE STANDING STANDING STANDING STANDING Heart rate (bpm) Blood pressure (mmHg) LYING LYING & SITTING SITTING SITTING Nocturnal Period 0 10:00 0 12:00 17:00 20:00 Time (hours) 00:00 07:00 10:00 Causes & Associations of PoTS Partial peripheral autonomic neuropathy Noradrenaline transporter deficiency Hypovolumeia PoTS Deconditioning Panic attacks Joint hypermobility syndrome Mitral valve prolapse Chronic fatigue syndrome Migraine Mast cell disorders PoTS – AMS A syndrome NOT a disease! PoTS – AMS Joint Hypermobility Syndrome Joint Hypermobility Syndrome Familial Connective tissue disorder • Hyperlaxity of the joint • Posturally induced headache • Subset with pelvic/visceral symptoms affecting upper and lower GI tract & urinary bladder • Vascular pooling in the periphery while standing What happens on standing? Joint Hypermobility Syndrome • Venous pooling in the periphery when upright PoTS and AMS Multidisciplinary approach Clinicians Autonomic Nurse Specialist 1) Clinical spectrum 2) Complete autonomic evaluation 3) Tailored management Autonomic Scientists Thank you for your attention Creating a culinary masterpiece Autonomic Management Plan The Ingredients • • • • • • • Expectations Identify symptom triggers Other medical conditions and medication Non-drug treatment Drug treatment Other health professionals Occupational health, colleges/universities, DVLA This will be unique for each individual Tailor made treatment The Recipe Add non-pharmacological measures Combine with medication if needed Non-pharmacological measures To minimise the effect of • Environment – gravity, heat • Daily activities – eating, exercise • Psyche – pain, anxiety Environmental • Preventing pooling • Activation Measures • Exercise - especially strengthening of leg musculature • Physiotherapy Daily activities • Salt addition • Fluid repletion • Small meals • Pace activity • Occupational therapy Fear • Information ,support Pain • Management programs Anxiety • Relaxation • CBT Pharmacological Mineralocorticoids - Fludrocortisone Sympathomimetics that do not raise heart rate Midodrine Cholinesterase inhibitors – Pyridostigmine Beta-adrenergic blockers, cardioselective Clonidine I(f) Channel blocker - Ivabradine Tachycardia v partnership autonomic team non-pharmacological medication = The Autonomic Clinic: Clinical Research Aspects Dr David Low, PhD The Autonomic Units’ Research Team Prof. C.J. Mathias Clinical & Research Director Dr David Low Dr Ekawat Vichayanrat Clinical Research Lead UKIERI Clinical Research Fellow Dr Valeria Iodice Dr Juan Carlos Sanchez-Manso Sir Roger Bannister Clinical Research Fellow EFNS Clinical Research Fellow William Seligman Andrew Owens MBBS Clinical Research Project Student Clinical Research Co-ordinator Plus others! The Autonomic Units’ Research Team Prof. C.J. Mathias Clinical & Research Director Research Team Clinical Team Fellows Students Co-ordinators Doctors Nurse Specialists Autonomic Scientists Administrative Team Research Aims • Improve diagnosis – define features of autonomic disease – develop novel non-invasive techniques • Understand causes of autonomic disorders – determine pathophysiological mechanisms • Devise and evaluate new treatments – improve management of disorders and their complications Research Achievements Detailed phenotyping of autonomic disorders Clinical and Research training placements Development of treatment strategies Diagnostic techniques for central & peripheral disorders Novel investigations of physiology/pathophysiology Autonomic Nervous System Function Autonomic Dysfunction Symptoms Blood Pressure Continence Reproductive Autonomic Dysfunction Gastrointestinal Temperature Sleep Autonomic Disorders Intermittent Primary/ Secondary • Syncope • Postural Tachycardia Syndrome (PoTS) • Acute/Chronic • Metabolic/Trauma/Drugs Autonomic Function Research Clinical Test Research Study Autonomic Function Research Autonomic Function Research Autonomic Dysfunction Blood Pressure Continence Reproductive Autonomic Dysfunction Gastrointestinal Temperature Sleep Postural Tachycardia Syndrome and AMS venous pooling hyperadrenergic tone physical deconditioning relative chronic hypovolemia genetics JHS/collagen dysfunction Familial PoTS/EDS III Family B Family A Family C I1 II1 III1 III2 III3 II2 II3 II 1 II1 II2 II 2 Temperature Skin Neurovascular Function Gastrointestinal Function Pacemaker area Autonomic Dysfunction and Exercise Training Research Process Develop Protocol Ethical Approval Data Collection Data Analyses Publication of Findings Research Process Develop Protocol Ethical Approval Data Collection Data Analyses Publication of Findings Recruitment Clinical & Research Fellows Dr David Low - Clinical Research Lead Dr Valeria Iodice - Sir Roger Bannister Clin.Res. Fellow Dr Ekawat Vichayanrat - Clinical Research Fellow Dr Juan Carlos Sanchez-Manso – Clin. Res. Fellow Clinical Autonomic Scientists Senior Clinical Autonomic Scientists Madeline Tippetts Katharine Bleasdale-Barr Lydia Mason Clinical Automomic Scientists Michael Peche Vanessa Ponnusamy Ian Skeavington O. Osiguwa Fiona Vaidya Helen Cannon Seema Maru Kiran Sheri Clinical Research Co-ordinator Andrew Owens Clinical NeuroendocrineScientist Laura Watson Consultant & Head of Department Prof. CJ. Mathias Consultant Dr. G. Ingle Autonomic Nurse Specialist Catherine Best Thank you for your attention Autonomic Function in Health & Disease