Syncope Common and Uncommon Causes Kelly Airey, MD, FACC, FHRS Cardiology and Electrophysiology October 1. 2016

Disclosures - Consustant/Speaker – Biosense Webster

Objectives Identify causes of syncope Identify differences between benign and malignant forms of syncope Identify signs and symptoms which help differentiate various types of syncope

Definition Syncope is a transient loss of consciousness (TLOC) resulting from global cerebral hypoperfusion, characterized by rapid onset, brevity and spontaneous recovery EHJ. 2009;30:2631-2671

What is the physiology? Regardless of cause: Syncope is usually the result of cerebral hypo-perfusion Pre-syncope is a less severe manifestation of the same process Reduced oxygen and glucose can mimic the same condition I tell my pts that in most cases, the cause is not fatal, it is the end result

Facts The most common cause of syncope regardless of age, sex or comorbidity is vasovagal. The second most common is cardiac. Carotid sinus and OH rarely occur in those under 40 years Circulation.2013;127:1330-1339

Orhostaic Hypotensive TLOC Cardiac Syncope Orhostaic Hypotensive Reflex Mediated Unknown Non-syncope

Intracranial hemorrhage Non-syncopal TLOC Neurologic Seizure Stroke or TIA (RAS) Intracranial hemorrhage Other Endocrine Hypoglycemia Psychiatric Pulmonary Embolism

Hypertrophic cardiomyopathy* Cardiac Syncope Obstructive Aortic stenosis Hypertrophic cardiomyopathy* Cardiac tumors Arrhythmia tachycarrhythmias bradyarrhythmia

Reflex Mediated Syncope situational vasovagal Carotid Hypersensitivity

TLOC - Common Causes

What does it cost? In the US, 30-40% are admitted at an annual cost of $2.4 billion dollars. This is related to multiple low yield investigation and unnecessary hospitalizations Circulation.2013;127:1330-1339

infrequent, unexplained: 38% to 47%1-4 Recurrent Syncope infrequent, unexplained: 38% to 47%1-4 explained: 53% to 62% 500,000 new syncope patients each year5 170,000 have recurrent syncope6 70,000 have recurrent, infrequent, unexplained syncope 1-4 Studies have shown that the cause of syncope remains undiagnosed in as many as 47% of the patients who present with this symptom. There are approximately 170,000 recurrent syncope patients in the U.S. today, meaning that up to 70,000 patients with recurrent, infrequent syncope may be going undiagnosed and therefore, improperly treated. Of these patients, 20,000 may have undergone extensive testing with no diagnosis. 1.Kapoor W. Evaluation and outcome of patients with syncope. Medicine 1990;69:160-175. 2.Silverstein M, et al. Patients with syncope admitted to medical intensive care units. JAMA 1982;248:1185-1189. 3.Martin G, et al. Prospective evaluation of syncope. Ann Emerg Med 1984;13:499-504. 4.Kapoor W, et al. A prospective evaluation and follow-up of patients with syncope. N Eng J Med 1983;309:197-204. 5.National Disease and Therapeutic Index, IMS America, Syncope and Collapse #780.2; Jan 1997-Dec 1997. 6.Kapoor W, et al. Diagnostic and prognostic implications of recurrences in patients with syncope. Am J Med 1987;83:700-708. 1 Kapoor W, Med. 1990;69:160-175. 2 Silverstein M, et al. JAMA. 1982;248:1185-1189. 3 Martin G, et al. Ann Emerg. Med. 1984;12:499-504. 4 Kapoor W, et al. N Eng J Med. 1983;309:197-204. 5 National Disease and Therapeutic Index, IMS America, Syncope and Collapse #780.2; Jan 1997-Dec 1997. 6 Kapoor W, et al. Am J Med. 1987;83:700-708.

Prognosis for syncope Prognosis is determined by the underlying etiology, specifically the presence and severity of cardiac disease. Untreated, can be >10% at 6 months NEJM, 2002:879-885

The Typical Syncopal Patient 61 years of age on average 55% are female 43% Vasovagal 14% Cardiac 43% other (including unknown) Canadian Cardiovascular society commissioned research to streamline assessment and treatment process

After the ER presentation 0.7% die in 30 days 10% die within one year 7.5% will have a non-fatal severe outcome in ER (new dx, clinical deterioration, serious injury with recurrence, require therapeutic intervention) 4.5% will have a non-fatal severe outcome within 7-30 days

What is important To identify and treat the 4.5% that will have a non-fatal severe outcome in the next 7-30 days after presentation. Only a small proportion are cardiac in nature

Benign syncope – Cause of syncope is has good prognosis and is not life-threatening (vasovagal and other reflex-mediated) Malignant syncope – Cause of syncope is life –threatening (structural heart disease, inherited conditions; tachy/brady arrhythmias)

“It’s not the fall that hurts…. It’s the sudden stop at the end… “It’s not the fall that hurts…. It’s the sudden stop at the end….” - Will Rodgers

Cause of Syncope Age and Risk Considerations Young Old Benign Vasovagal Situational Psychogenic Orthostatic - drugs - multifactorial Malignant Inherited/Congenital disorders causing tachycarrhythmias - long QT - CPVT - ARVC - HCM - DCM Arrhythmias - bradarrhythmias - tachycarrhythmias

17 year old male presents after passing out at 4 am while urinating after waking up from a sound sleep. He was diaphoretic at the onset of urination. He woke up on the ground in the bathroom. He was alert and oriented. Long standing history of early morning light-headed episodes and syncope as a child while standing in church.

17 year old male passed out at football practice 17 year old male passed out at football practice. He had just run 100 yards and started feeling light headed at 50 yds. He collapsed to the ground shortly after. Witnesses claimed that he just fell and was unresponsive for 30 seconds after with complete recovery. No seizure noted. No previous medical history.

64 year old woman with long standing, poorly controlled hypertension passed out in the kitchen after gardening in 95 degree temperatures for three hours. EKG shows LVH. Last echo showed normal EF. Medications include lisinopril/hctz; metoprolol; furosemide; asa.

63 year old woman passed out while sitting in a chair 63 year old woman passed out while sitting in a chair. History of anterior MI six months earlier with occluded LAD that couldn’t be opened. Class II-III heart failure symptoms with EF of 35% with anterior hypokinesis. EKG shows old anterior infarct. Medications included metoprolol, lisinopril, ASA, furosemide.

Hypertrophic cardiomyopathy* Cardiac Syncope Obstructive Aortic stenosis Hypertrophic cardiomyopathy* Cardiac tumors Arrhythmia tachycarrhythmias bradyarrhythmia Reflex Mediated situational vasovagal postural hypotension

Vasovagal Syncope Syncope Upright posture Venous pooling Decreased venous return Increased adrenergic stimulation Increased LV contractility Increased LV mechanoreceptor activity Vagal Afferent activity Medullary Cardioinhibitory Center vagal sympathetic sympathetic vagal Sinus node deceleration Arterial hypotension bradycardia Syncope hypotension

Pathophysiological basis of the classification of reflex syncope. ANF Pathophysiological basis of the classification of reflex syncope. ANF . autonomic nervous failure; ANS . autonomic nervous system; BP . blood pressure; low periph. resist. . low peripheral resistance; OH . orthostatic hypotension. EHJ 2009;30:2631-71

Other Reflex Syncopes Carotid hypersensitivity Postural Tachycardia Syndrome (POTS) Primary autonomic failure

Situational Syncope Variant of vasovagal syncope Recognized trigger or situation Causal link not clear (?reflex arc) Examples include: micturition, cough, defecation, visiting the hospital

Arrhythmias that Cause Syncope VF Polymorphic VT/Monomorphic VT AV Block Sinus bradycardia/pause Hypotensive SVT

VF/VT Result of any one of a number of acquired or inherited disorders Look for clinical clues to suggest the patient at risk for VT/VF

Causes of VT/VF Inherited Catecholaminergic polymorphic VT Long QT syndrome Brugada Syndrome Hypertrophic Cardiomyopathy Dilated Cardiomyopathy WPW Aquired Ischemic Cardiomyopathy Non-ischemic Cardiomopathy Hypertensive Alcoholic Fibrosis Infiltrative

822 patients with syncope from the Framingham cohort (7814 total) followed for 17 yrs

Major – more than one study – minor – one study only One major or >1 minor - should see cardiovascular specialist within two weeks

Clues to Suggest Malignant Cause Lack of prodrome History of heart disease (CAD, valvular disease, CM) Family history of early SCD Syncope during exercise Abnormal EKG (WPW, Brugada, AV conduction disease, BBB)

Syncope and Age

Clinical Clues TLOC

EKG abnormalities …any syncopal patient with an abnormal EKG needs further investigation

Syncope work up

17 year old male presents after passing out at 4 am while urinating after waking up from a sound sleep. He was diaphoretic at the onset of urination. He woke up on the ground in the bathroom. He was alert and oriented. Long standing history of early morning light-headed episodes and syncope as a child while standing in church.

17 year old male passed out at football practice 17 year old male passed out at football practice. He had just run 100 yards and started feeling light headed at 50 yds. He collapsed to the ground shortly after. Witnesses claimed that he just fell and was unresponsive for 30 seconds after with complete recovery. No seizure noted. No previous medical history.

64 year old woman with long standing, poorly controlled hypertension passed out in the kitchen after gardening in 95 degree temperatures for three hours. EKG shows LVH. Last echo showed normal EF. Medications include lisinopril/hctz; metoprolol; furosemide; asa.

63 year old woman passed out while sitting in a chair 63 year old woman passed out while sitting in a chair. History of anterior MI six months earlier with occluded LAD that couldn’t be opened. Class II-III heart failure symptoms with EF of 35% with anterior hypokinesis. EKG shows old anterior infarct. Medications included metoprolol, lisinopril, ASA, furosemide.

Thank you!