Syncope. enough to include disorders such as epileptic seizures and concussion. January 14-15, 2011 SCA Conference 1

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1 Syncope and Atypical Seizures Ravi Mandapati, M.D., FACC.; FHRS Director, Specialized Program for Arrhythmias in Congenital Heart Disease UCLA Cardiac Arrhythmia Center David Geffen School of Medicine at UCLA Director, Pediatric Cardiac Electrophysiology Loma Linda University Medical Center Syncope Syncope is a transient loss of consciousness sness due to transient global cerebral hypo perfusion characterized by rapid onset, short duration, and spontaneous complete recovery. Without qualifying transient global hypo perfusion, the definition of syncope becomes wide enough to include disorders such as epileptic seizures and concussion. January 14-15, 2011 SCA Conference 1

2 Seizures are frequently and inappropriately classified as syncope Seizures Sheldon et al. Diagnosis of Syncope and Seizures, JACC 2002 January 14-15, 2011 SCA Conference 2

3 Seizures Sheldon et al. Diagnosis of Syncope and Seizures, JACC 2002 Breath holding spells Infantile reflex syncopal attacks or pallid breath holding spells elicited by noxious stimuli are caused by vagally mediated cardiac inhibition. Cyanotic breath holding spells that occur with expiratory cessation of respiration during crying January 14-15, 2011 SCA Conference 3

4 Neurally Mediated Reflex Syncope/Triggers Emotion/pain Prolonged standing Micturition Post exercise Hyperventilation and straining Stretching Coughing Standing up suddenly Deglution Syncope : Mechanical /Structural Aortic Stenosis Hypertrophic cardiomyopathy Anomalous coronary artery Severe pulmonary hypertension January 14-15, 2011 SCA Conference 4

5 Syncope : Rhythm Disturbances Brady arrhythmia Sinus node dysfunction AV conduction disease Kearns Sayre syndrome (external ophthalmoplegia and progressive heart block), CHB Device malfunction Tachyarrhythmia AF in WPW SVT ( with HR > 250/min and LV dysfn, rare) VT Inherited arrhythmia Long QT, Brugada, CPVT, ARVD, early repolarization Syncope : Other Causes Cerebrovascular Vascular steal syndromes Non Syncopal attacks Metabolic disorders( hypoglycemia, hypoxia, hyperventilation-hypocapnia) hypocapnia) Epilepsy TIA Somatization disorders January 14-15, 2011 SCA Conference 5

6 3 year old Case # 1 3 episodes of seizures PDA, s/p ligation Syndactyly Mild developmental l delay January 14-15, 2011 SCA Conference 6

7 Timothy Syndrome QT=450 ms QTc=QT/ RR=580 ms Case # 1 :What should be done? 1. Beta blocker 2. Pacemaker 3. AICD 4. Sympathectomy January 14-15, 2011 SCA Conference 7

8 Self termination of VF/Torsades Initiation of torsades Self termination ICD shock terminates Torsades Torsades detected Redetection 31 J ICD shock January 14-15, 2011 SCA Conference 8

9 Follow Up Implanted AICD and sympathectomy Did well till age 5 with occasional shocks One episode of VF storm Pocket infections after placing rate sensing lead Transplant Renal dysfunction Case # 2 16 year old, active Palpitations Syncope x1 Brought to ER with tachycardia, near syncope January 14-15, 2011 SCA Conference 9

10 Case # 2 : What is the diagnosis? 1. Ventricular tachycardia 2. Ventricular fibrillation 3. Atrial fibrillation 4. Atrial fibrillation in WPW Antegrade conduction of SVT over accessory pathway: a fib Atrial Fibrillation varying degree of fusion RR intervals irregularly irregular January 14-15, 2011 SCA Conference 10

11 Case # 3 17 yr old High school star basketball player Syncope during practice 3 rounds of CPR by coach Seizures after CPR Full neurological recovery Echo HCM January 14-15, 2011 SCA Conference 11

12 Case # 3 : what should be done? 1. Neuro consult 2. Beta blocker 3. EP Study, AICD if positive 4. AICD Follow Up AICD Single zone VF : 210 beats /min Second opinion for sports Inappropriate shock few days ago January 14-15, 2011 SCA Conference 12

13 Case # 4 16 year old Multiple episodes of syncope Possible seizure during one episode Most episodes are posture related In corrections facility Telemetry strips at outside facility January 14-15, 2011 SCA Conference 13

14 Telemetry strips at LLU Case # 4 : what will you do? 1. Tilt table test 2. Manage as vasovagal syncope 3. EP study and ablation of PVC s 4. Genetic testing / external jacket 5. AICD January 14-15, 2011 SCA Conference 14

15 Follow Up Implanted AICD after much discussion/consultation VV syncope controlled on florinef Expect multiple hospital/er visits Case # 5 9 year old trisomy 21 s/p VSD repair Complete heart block Epicardial pacemaker, gen change x1 Presented with seizure like episode Brought to ER at 11 PM, pale lethargic EF 20 % (prev normal function) January 14-15, 2011 SCA Conference 15

16 Case # 5 : what will you do? 1. Atropine 2. Isuprel drip 3. Epinephrine drip 4. External pacing 5. Temporary pacing wire 6. Implant transvenous permanent pacemaker Follow Up Implanted dual chamber pacemaker January 14-15, 2011 SCA Conference 16

17 8dayold Case # 6 Seizure, arrest at home, CPR by dad Abnormal echo Documented VF in hospital, defibrillated Echo Open epicardial Bx: rhabdomyoma January 14-15, 2011 SCA Conference 17

18 Case # 6: What will you do? 1. Beta blocker 2. Amiodarone 3. Surgical resection 4. AICD 5. Cardiac Transplant January 14-15, 2011 SCA Conference 18

19 Follow Up AICD at age 1 month DC home on amiodarone and propranolol One episode of non sustained VF recorded on device Progressive lung compression by tumors, Resp distress CT guided Bx confirmed rhabdomyoma Heart Tx at age 6 months Syncope - History Most important aspect of syncope evaluation Onset? t?sudden suggests arrhythmia Prodrome? If yes, suggests neurocardiogenic Positional? Yes, then orthostatic Supine? More concerning for arrhythmias Preceding events? Swallowing/cold water/urination rination suggest neurocardiogenic Witnesses? May provide best history January 14-15, 2011 SCA Conference 19

20 High risk criteria which require intensive evaluation Severe LV dysfunction Syncope during exertion or supine Palpitations Family h/o SCD Non-sustained VT WPW Long QT/Short QT Brugada pattern ARVD pattern Important co-morbidities Severe anemia Electrolyte disturbances January 14-15, 2011 SCA Conference 20