Beware the Red Flags in the Management of Syncope
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1 Beware the Red Flags in the Management of Syncope Mitchell Cohen MD FACC FHRS Co-Director of the Pediatric Heart Program Director of Pediatric Arrhythmia Services Inova Fairfax Children s Hospital President, Pediatric & Congenital Electrophysiology Society
2 Disclosures: None I am going to discuss Red Flags in clinical scenarios of children and adolescents after an initial presentation of syncope that presented to me.
3 Is it this.. Or that..
4 Initial Presentation of Syncope My View No Worry Mild Worry Moderate Worry Severe Worry
5 February year old male presents with syncope Playing basketball game towards end of game dizzy; weak sat down on half-court line slumped unconscious pale, no tonic-clonic movements admitted to ER; received IV hydration discharged finished out basketball season no problems Case #1
6 December 2008 varsity basketball running sprints - collapsed
7 no left ventricular hypertrophy no wpw no qt prolongation no brugada
8 Stress Test: normal no symptoms maximum heart rate 208 bpm maximum blood pressure 166/96 mmhg
9 PA Ao Anomalous Left Coronary Artery From Right Facing Sinus
10 Normal Left Coronary Artery Origin
11 Syncope with Exertion DO NOT STOP UNITL YOU FIND A CAUSE
12 Case #2 4 year-old girl who presents for a heart murmur On the clinic note indicated that she has had two syncopal events? The most recent episode occurred when she was standing at the window, she saw dad pulling into the garage and fainted. Mom thought she just tripped.
13 Episode #2: standing in the kitchen, eating a banana when the phone rang and she fell forward. Mom thought she was just playing as she was back to normal a few seconds later. Examination: Normal Family History: Negative
14 QTc 560 Long QT Type II
15 Syncope with Exertion Syncope with Sudden Noise
16 a miss Case #3 7 y.o. boy new-onset seizure while running at recess Admitted neurology, diagnosed with a seizure disorder no EKG no cardiology consult is obtained family history is negative for sudden death discharged the following day on a seizure medication 6 months later drowns in the swimming pool. the 5 y.o. brother is referred for evaluation ekg on arrival of the 5 year-old brother:
17 EKG: Long QT & T wave alternans
18 when I met with the family I had asked the parents if any close relatives had died under 30 y of age. The answer was No when I asked the dad how many brothers and sisters he has he told me: 2 brothers and 1 sister. He had a brother that died before he was born how did the brother die? (swimming age 5, but no one talks about it ) the dad s EKG was also c/w LQT
19 Long QT Syndrome Inherited disease (most autosomal dominant) Prolongation of cardiac repolarization Longer time for the heart to relax Genetic Basis Cardiac ion (potassium, sodium, calcium) channel disease LQT 1, 2, 3 account > 85-90% all LQT cases
20 Channelopathies Diseases that affect cardiac ion channels LQTS prototype channelopathy
21 Cardiac Action Potential
22 abnormal potassium channel Sodium (Na + ) goes in Potassium (K + ) cannot leave Cell has more (+) charge=cell excited and cannot relax
23 Clinical Manifestations of LQT None Fainting Palpitations Sudden cardiac death
24 Clues to LQT Syndrome Personal or Family History of: Stress induced fainting / palpitations Stress induced seizures (1/3 of LQT misdiagnosed as epilepsy) Unexplained drowning / Near drowning Unexplained motor vehicle accidents Family History of: Unexplained Sudden Death <40 y/o SIDS
25 Percent Occurrence of Gene-Specific Triggers LQT 1 LQT 2 LQT 3 LQT1 LQT2 LQT3 EXERCISE EMOTION REST Circ 2001;103:89-95
26 how good you are on obtaining a family history is only as good as the effort you put in
27 BACK TO OUR EKG QTc 640 ms patient clearly has LQT why gene test?
28
29 Father Mother Sister
30 Careful in the Waiting Room
31 Syncope with Exertion Syncope with Sudden Noise Syncope with a Concerning Family History
32 Case #4 13 year old boy presents to the hospital with a syncopal event while playing soccer on a typical Arizona summer day He was dizzy running down field and fainted. Fell hit head, small subdural hematoma Admitted to Neurosurgery Service
33 Left tempoparietal hemorrhage
34 an astute medical student. Met family and found out that this is the 3 rd syncopal event that occurred while playing soccer but the other two episodes it was not hot outside. Time for a Cardiology Consultation According to the resident exam normal, ECHO normal, no other concerning elements Resident Request: You just need to clear (is he OK) him before anesthesia takes him down for another MRI
35 A few more questions Was he well when he had these passing out episodes? Well he had a fever all three times Let s go through the family history one more time, let s go through each of your siblings Uncovered that dad s brother died suddenly at age 15 in the midst of a fever- it was attributed to a viral infection of the heart.
36
37 Brugada Syndrome Inheritable electrical disease Characterized by a typical ECG pattern Structurally normal heart Presents with syncope, sudden death, seizures due to polymorphic ventricular tachycardia Adolescents and young adults
38 Syncope with Exertion Syncope with Sudden Noise Syncope with a Concerning Family History Syncope With a Fever
39 Case #5 9 year old: 6 month history of exercise intolerance, chest pain, and a recent syncopal episode playing soccer Moved from midfield to striker to goalie, so he can stand there.
40 12 Lead Electrocardiogram
41
42 42 segments of coronary narrowing Started beta-blockers Placed dual chamber ICD Referred for transplant evaluation
43 Hypertrophic Cardiomyopathy Uncommon ( % general population) Most common cause of sudden death in young Mutations in genes encoding sarcomere Sudden death may be 1 st symptom Variably hypertrophied (mild to massive) Autosomal dominant Variable penetrance
44 Hypertrophic Cardiomyopathy
45 Symptoms of Hypertrophic Cardiomyopathy Tremendous variability in symptoms No symptoms Palpitations Dizziness Passing out Exercise intolerance Sudden cardiac death
46 Hypertrophic Cardiomyopathy Treatment Beta blockers and Calcium channel blockers to reduce HR and contractility Avoid dehydration AICD if septal thickness significant or symptomatic Muscle resection for relief of LV obstruction SCD prevention by diagnosing before disease manifests (genetic testing) Exercise restrictions is very effective
47 Syncope with Exertion Syncope with Sudden Noise Syncope with a Concerning Family History Syncope with a Fever Syncope with Chest Pain
48 Adolescent with a 1 month history of palpitations (appointment scheduled with cardiology next week) Syncope During Dodge Ball, Connected to AED Case #6
49 12 Lead EKG after EVENT
50 Syncope with Exertion Syncope with Sudden Noise Syncope with a Concerning Family History Syncope with a Fever Syncope with Chest Pain Syncope with Palpitations
51 7-year-old boy brought to the cardiology office after a vague syncopal episode while playing soccer. Case #7
52 Fall 2006 No previous syncopal events while running. Baseline EKG normal sinus rhythm Echo: Normal No family history of syncope or SCD
53 Peak Exercise bidirectional couplets Started on beta-blockers- repeat EST unchanged Added calcium-channel blockers- repeat EST unchanged
54 3 weeks later while running to 2 nd grade class, collapses in front of school nurse s office- (school has an AED) Catecholaminergic Polymorphic VT
55 Catecholaminergic Polymorphic VT Polymorphic (bidirectional) VT in children Structurally normal heart Presents > age 3 years Syncope often thought to be seizures Uncontrolled release of calcium sets up life-threatening arrhythmias 1/3 family history of syncope or sudden death Sudden death during adolescence Mutations RyR2 cardiac ryanodine receptor
56 Natural History of CPVT 67% of patients have exercise/emotional fainting 33% sudden cardiac death 80% of patients will have symptoms < 40 years One of the most severe arrhythmia disorders
57 Treatment of CPVT Beta-blockers are the mainstay of treatment If fainting persists or there are continued ventricular arrhythmias despite betablockers an ICD is warranted
58 Syncope with Exertion Syncope with Sudden Noise Family Histories Often Provide A Clue Syncope with a Fever Syncope with Chest Pain Syncope with Palpitations Even Being Aware of Red Flags: Secondary Precautions are Important
59 Know Where the AED Is Located! Where Did You Say the AED was??
60 Syncope with Exertion Syncope with Sudden Noise Family Histories Often Provide A Clue Syncope with a Fever Syncope with Chest Pain Syncope with Palpitations Even Being Aware of Red Flags: Secondary Precautions are Important Syncope with NO SYMPTOMS
61 Goals of Assessment of Syncope 1. Evaluate for underlying heart diseases that alter ion channel function cardiac myocyte function intracellular matrix 2. Determine safety of returning to sports 3. Who else in family needs to be tested 4. Reassurance 5. Therapy if needed
62 Prove Vasovagal Syncope Not... Vasovagal syncope is transient loss of consciousness inability to maintain postural tone spontaneous recovery absence of.. seizure arrhythmia heart disease
63 Vasovagal Syncope Detailed History of Event Predisposing situations Prodromal symptoms Physical signs Recovery time and symptoms
64 Predisposing Situations Prolonged standing, sitting, position change Medical, painful, yucky
65 Prodromal Symptoms Warmth Nausea Clouded thinking Vision/hearing changes Tunnel Like Vision Dizziness Syncope without prodrome or with injury is worrisome
66 Prodrome May be falsely reassuring > ½ patients with channelopathy reported a prodrome before a syncopal event MacKormik Heart Lung Circulation 2011
67 Physical Signs Pallor Diaphoresis Hypotension Relative bradycardia Myotonic movements may be interpreted as seizure
68 Recovery Time and Symptoms After Unconsciousness for < 2 minutes, full recovery sluggish Tired for minutes-hours after Headache Careful and focused history makes the diagnosis
69 Personal/Family History Personal History Previous syncope/dizziness Palpitations Chest pain Drugs including prescribed, illicit, supplements and sports enhancing Family History Sudden death Known heart disease SIDS Drowning Recurrent fetal loss
70 Testing? History/Physical ECG History/Physical ECG Echo Exercise test History/Physical ECG Echo Exercise test Tilt Holter EP Study Loop recorder MRI Genetic testing
71 Case #8 16-year-old boy faints at the end of the high school state cross country finals. Elite athlete headed for scholarship to an Ivy League No chest pain and no palpitations I was getting a little dizzy before the finish line went black Self-recovered Cool outside Pushing himself super hard
72
73 I Saw This A Lot!!!!!
74 Why do endurance athletes faint? Abnormal BP response - depressed carotid baroreceptor sensitivity Increased stroke volume, more compliant, distensible ventricles - chronic volume overload delivery large volume of blood to muscles Disadvantage during orthostasis Results in large decrease in SV when filling pressure reduced Whyte Br J Sports Medicine 2004
75 My Approach to the Exercise Syncope (syncope before the finish line) with No Easy Answer Assuming normal exam and family history ECG- Normal ECHO Normal Stress Test Normal Holter No concerning PVC burden, normal CT scan or MRI (coronaries unless echo 100%) Implantable loop recorder (if truly exertional) Resume competition (must have AED available, my advocacy) Treatment: Vasovagal approach
76 Management of Vasovagal Syncope Be Aware of Situations Hydration, salt, support stockings No other therapy unless syncope persists, danger of injury or drowning pharmacotherapy midodrine, florinef 2017: beta-blockers Lower body (+) pressure maneuvers after exercise may limit/eliminate events
77 Thank You
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