Diagnosis and Management of Syncope Robert Helm M

Diagnosis and Management of Syncope Robert Helm, M. D. Assistant Professor of Medicine Boston University School of Medicine August 2013

Case 1 53 year-old obese gentleman with diabetes, hypertension and hyperlipidemia who presented with syncope. This occurred after working at the boat yard on a very hot day. He was taking a break and drinking a cold slurpee, when he suddenly felt pins and needles in his neck, peri-oral numbness, and tingling of his forehead. As he was calling for wife, he lost consciousness and fell to the ground. According to his wife, he was unconscious for about a minute and his entire body was quivering. Upon regaining consciousness, he was confused and disoriented. His wife reports that he was cold, clammy, and very diaphoretic. He sustained some minor bruising of left shoulder but no head injuries.

Case 1 No prior syncope but two weeks ago he did a “pirouette” due to sudden brief episode of lightheadedness and loss of balance. He did not lose consciousness. He consulted with his internist, who found relative low blood pressure (100/54 mm. Hg) and reduced his Lisinopril by half (40 to 20 mg daily). His blood sugars have been well controlled.

Case 1 Past Medical History Diabetes (Hg. BA 1 C 5. 8) Hypertension Hyperlipidemia ? myocardial infarction Obstructive sleep apnea Allergies None Medications Cardizem cd 120 mg daily Lisinopril 20 mg daily Lantus 40 units at night Social History Novalog 12 units with meals Non-smoker Aspirin 81 mg daily No alcohol or drugs Lasix 80 mg daily Simvastatin 80 mg at night Family History Fenofibrate 134 mg daily Mother had MI at 60 Percocet 5/325 mg as needed for pain Brother died suddenly at 48 Viagra 100 mg – last used 2 days prior Nitroglycerin 0. 4 mg SL – never used Review of system Negative. Good functional capacity.

Case 1 Physical examination BP 124/62 mm. Hg. Not orthostatic. Normal carotid palpation and auscultation. Normal cardiovascular exam. Bruised right hip. Laboratory BUN 27 ng/dl Creatinine 1. 47 ng/dl. Electrolytes and blood count normal. Echocardiogram – normal 1 month ago.

Case 1

Case 1 Admitted for 24 hour observation and hydration. Diagnosis: “Vaso-vagal syncope”

Poll The correct statement is: A. The patient has been correctly diagnosed. B. The patient should be referred for urgent pacemaker. C. The patient should be referred for electrophysiologic study. D. The patient should be referred for 30 day event recorder. E. The patient should be referred for tilt-table study.

Case 2 61 year old gentleman with history of cocaine use who presented with syncope. “He was in kitchen making Thanksgiving dinner and developed lightheadedness. He sat on a step stool and the next thing he remember is being on the floor and his son calling his name. He felt like he couldn’t move. The kitchen was really hot and he had missed lunch. He reports using cocaine “months ago”.

Case 2 Past Medical History none Allergies None Social History history of cocaine Medications None Family History no premature CAD or SCD Review of system Negative.

Case 2 Physical examination BP 145/91 mm. Hg. Not orthostatic. Normal carotid palpation and auscultation. Normal cardiovascular exam. Minor bruise on elbow. Laboratory BUN 26 ng/dl Creatinine 1. 1 ng/dl. Electrolytes and blood count normal.

Case 2

Poll The correct next step is: a. Check toxicology screen. If positive then attribute syncope to cocaine use. b. Continuous-loop event monitoring. c. Increase fluid intake and reassure patient. d. Electrophysiology study to assess for inducible VT e. Tilt-table study. f. Implant loop recorder (ILR). g. Echocardiogram. h. B and then D if event monitoring is negative. i. B and then F if event monitoring is negative. j. G and if ejection fraction is < 35% then D

Why is syncope a difficult problem? • Physiologic response to a wide variety of medical conditions • By definition it is a transient condition • Occurs with unpredictable and random pattern • Difficult to establish definitive “diagnosis” • “Another patient with syncope…. ” • History from patient may not be reliable.

Amnesia for Loss of Consciousness in Carotid Sinus Syndrome Falls (n=34) Syncope (n=34) P value 5. 1 5. 4 0. 42 Right Positive CSM 24 (71%) 29 (85%) 0. 92 CSM positive upright 20 (59%) 9 (26%) 0. 24 LOC during CSM 22 (64%) 15 (44%) 0. 144 Amnesia for LOC 21 (95%) 4 (27%) <0. 001 Mean max asystole (s) Perry S, et al: J Am Coll Cardiol 2005; 45: 1840

Causes of Syncope Neurally mediated reflex syncopal syndromes Vasovagal (common) faint Carotid sinus syndrome Situational faint Acute hemorrhage Cough, sneeze Gastrointestinal stimulation (swallow, defecation, visceral pain) Micturition (postmicturition) Postexercise Other (e. g. brass instrument playing, weightlifting, postprandial) Glossopharyngeal and trigeminal neuralgia Orthostatic Primary autonomic failure syndromes (e. g. pure autonomic failure, multiple system atrophy, Parkinson’s disease with autonomic failure) Secondary autonomic failure syndromes (e. g. diabetic neuropathy, amyloid neuropathy) Volume depletion Hemorrhage, diarrhea, Addison’s disease Cardiac arrhythmias as primary cause Sinus node dysfunction (including bradycardia/tachycardia syndrome) AV conduction system disease Paroxysmal supraventricular and ventricular tachycardias Inherited syndromes (e. g. long QT syndrome, Brugada syndrome, short QT, arrhythmogenic dysplasia) Implanted device (pacemaker, ICD) malfunction Drug-induced proarrhythmias Structural cardiac or cardiopulmonary disease Cardiac valvular disease Acute myocardial infarction/ischemia Obstructive cardiomyopathy Atrial myxoma Acute aortic dissection Pericardial disease/tamponade Pulmonary embolus/pulmonary hypertension Cerebrovascular Vascular steal syndromes

Classification of Syncope Common and benign Orthostatic Neurocardiogenic Common and not so benign Sinus node dysfunction, carotid sinus hypersensitivity Paroxysmal AV block Less common, lethal Ventricular tachycardia, ventricular fibrillation Torsade de pointes Everything else

Emergency Visits with Syncope European Society of Cardiology Guidelines 465 patients Number Percent Neurally mediated 309 66 Orthostatic Hypotension 46 10 Cardiac Arrhythmias 53 11 Cardiovascular 21 5 Unknown 11 2 Non-syncopal attack 25 5 Cause Brignole M, et al. European Heart Journal 2006; 27: 76 -82

Neurally - mediated Reflex syncope Vasovagal Carotid sinus hypersensitivity Situational Post-exercise Glossopharyngeal and trigeminal neuralgia Orthostatic syncope Primary autonomic failure Secondary autonomic failure Volume depletion Drugs and alcohol

Reflex Mechanism - Bezold Jarisch Trigger Venous return BP Vagal efferent Small ventricle Sympathetic tone Reflex Syncope C-fibers Wall stretch Inotropy Contractility Arterial tone HR Vagal afferent Vasodilation BP Sympathetic withdrawal BP Chang-Sing P. Cardiol Clinics. 1991; 9(4): 641 -651

When is History and Physical Sufficient • Young patient with single presentation or clear situational dependency • Normal physical examination • Normal ECG • No significant injury • Low risk occupation

What about the rest of the patients? History & physical exam including CSM ECG Tilt table test Echocardiogram Electrophysiology study Holter monitor / Event recorder / Implantable Loop Recorder (ILR) Neurological evaluation Psychiatric evaluation

Role of history in differentiating NMS from cardiac syncope 341 patients Warm Place Abdominal Discomfort Weakness Feeling warm Awareness about to faint Yawning Syncope while standing Nausea Feeling tired Standing in one place Vomiting Feeling cold Lightheadedness Prodrome History > 4 yrs Alboni P, et al: J Am Coll Cardiol 2001; 37: 1921 -28

Role of history in differentiating NMS from cardiac syncope 341 patients Abdominal Discomfort Standing in one place Feeling cold History > 4 yrs Alboni P, et al: J Am Coll Cardiol 2001; 37: 1921 -28

Role of history in differentiating NMS from cardiac syncope 191 patients with cardiac disease Warm Place Abdominal Discomfort Weakness Feeling warm Awareness about to faint Yawning Syncope while standing Nausea Feeling tired Standing in one place Vomiting Feeling cold Lightheadedness Prodrome History > 4 yrs Alboni P, et al: J Am Coll Cardiol 2001; 37: 1921 -28

Role of history in differentiating NMS from cardiac syncope 191 patients with cardiac disease History > 4 yrs Alboni P, et al: J Am Coll Cardiol 2001; 37: 1921 -28

Case 2 53 year-old obese gentleman with diabetes, hypertension and hyperlipidemia who presented with syncope. This occurred after working at the boat yard on a very hot day. He was taking a break and drinking a cold slurpee, when he suddenly felt pins and needles in his neck, peri-oral numbness, and tingling of his forehead. As he was calling for wife, he lost consciousness and fell to the ground. According to his wife, he was unconscious for about a minute and his entire body was quivering. Upon regaining consciousness, he was confused and disoriented. His wife reports that he was cold, clammy, and very diaphoretic. He sustained some minor bruising of left shoulder but no head injuries.

Case 2

ECG – Abnormal conduction Prolonged PR / heart blocks Functional Short PR Structural Bundle branch blocks Vagal tone AV nodal or His Purkinje fibrosis AV node Medications Long QT His Short QT Mitral annular calcification Infiltrative Genetic AH HV Intra-cardiac recording PR interval

What about the rest of the patients? History & physical exam including CSM ECG Tilt table test Echocardiogram Electrophysiology study Holter monitor / Event recorder / Implantable Loop Recorder (ILR) Neurological evaluation Psychiatric evaluation

Tilt table test

Tilt Table Response consistent with NMS Pretest 1 min 12. 5 min Recovery ECG Syncope Blood Pressure (mm. Hg) Tilt 0 70 HR (BPM) 77 94 40 46 BP (mm. Hg) 115/70 125/80 55/30 98/55 Sra JS. Ann Intern Med. 1991; 114: 1013 -1019.

Echocardiogram • Strongly consider for all patients • Screen for hypertrophic cardiomyopathy • Stratification for EP study Ejection fraction < 30% - meet criteria for ICD 35 -50% - test for inducibility of VT

Electrophysiology Study • Risk stratification of ventricular arrhythmias – assess for inducibility • It is poor at diagnosing bradycardic arrhythmias • It is highly sensitive for tachycardias.

Holter Monitor Yield: Arrhythmia with symptoms = 2% Symtoms without arrhythmia = 15% Gibson TC et al Am J Cardiol 1984; 53: 1013 -17

Comparison of Loop Recorders versus Holter Monitor (COLAPS) Sivakumaran S, et al. Am J Med 2003; 115: 1 -5

Event recorder in patient with syncope

Implantable Loop Recorders (ILR) ILR Automatically detects bradycardia Records rhythm at tachycardia time of trigger asystole Patient Assist Device

ILRs

Example tracing from ILR

ILR in unexplained syncope with normal conventional work-up Tachycardia Asystole / bradycardia 11% 56% 33% No arrhythmia Diagnostic yield: 35% (175/506 patients) Brignole et al. Europace 2009; 11, 671 -687

Suspect Pacemaker malfuction 1. EKG 2. Interrogate pacemaker – check lead integrity with provocative maneuvers 3. Chest X-ray

Importance of Interrogating PPM or ICD Atrial lead Ventricular lead

2 Right ventricular lead Atrial lead

Syncope – red flags A. B. C. D. E. Syncope resulting in injury Syncope during exercise Syncope in the supine position Suspected or known structural heart disease ECG abnormality Pre-excitation (WPW) Long QT Bundle-branch block HR<50 bpm or pauses > 3 seconds Mobitz I or more advanced heart block Documented tachyarrhythmia Myocardial infarction F. Family history of sudden death G. Frequent episodes (>2 per year) H. Implanted pacemaker or defibrillator I. High risk occupation (bus driver, pilot etc. )� e c n a v d A e g A d

Case 1 - review Discharged after 24 observation with diagnosis of “Vaso-vagal syncope” 2 days later… Witnessed collapse while seated. Episode of syncope with complete heart block noted on telemetry. Dual chamber pacemaker implanted. Discharged home the next day.

Case 1 Suspected or known structural heart disease - prior MI Abnormal EKG – trifasicular block Family history of sudden death – brother died at 45 Frequent episodes – “pirouette” 2 weeks prior

Case 2 - review Discharged after 24 observation with diagnosis of “Vaso-vagal syncope” 3 month later. . Cardiac arrest at home and successfully defibrillated but prolonged down time. Had slow neurologic recovery. ICD implanted for secondary prevention.

Case 2

Test questions Which of the following historical findings are useful for predicting neurally-mediated syncope in patients with heart disease and recurrent syncope? a. Feeling warm. b. Awareness of being about to faint. c. Recovery duration lasting longer than 60 minutes. d. Confusion during recovery. e. Time (years) between first and last syncopal episodes.

Which of the following historical findings are useful for predicting neurally-mediated syncope in patients with heart disease and recurrent syncope? a. Feeling warm. b. Awareness of being about to faint. c. Recovery duration lasting longer than 60 minutes. d. Confusion during recovery. e. Time (years) between first and last syncopal episodes.

An 80 year-old frail woman presents to you after falling while ambulating to the bathroom at night. This is the second time she has fallen in the last month. Echocardiogram shows diastolic dysfunction and moderate mitral annular calcification. ECG is essentially normal with exception of first degree AV block. After her first fall one month ago, she was told by her physician that she needs to use a cane and to rise slowly out of bed. Her daughter is very concerned and wants a second opinion. You recommend which of the following: a. Discontinuing evening dose of Lasix. b. Tilt-table study to diagnosis the etiology of falls and reassure her daughter. c. Electrophysiologic test to assess for bradyarrhythmias. d. Continuous-loop event monitoring.

A 16 year-old girl presents to you after a syncopal event while playing field hockey on an unusually hot day. She was running when she developed profound lightheadedness just prior to losing consciousness. Upon regaining consciousness, she was diaphoretic and confused to surroundings. She felt nauseated for the rest of the day. She has no cardiac history and her exam is unremarkable except for mild orthostasis. Her EKG is normal. You recommend which of the following: a. Genetic testing for long QT channelopathy. b. Continuous-loop event monitoring. c. Increasing fluid intake and reassure parents. d. Refer to electrophysiology. e. Tilt-table study.