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 • Sy ncope is a transient loss of consciousness d ue 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 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 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 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 Syncope : Rhythm Disturbances • Brady arrhythmia – Sinus node dysfunction – AV con duc tion 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) – Epilepsy –TIA – Somatization disorders •January 14-15, 2011 SCA Conference •5 Case # 1 • 3 year old • 3 episodes of seizures • PDA, s/p ligation • Syndactyly • Mild d eve lopment al d el ay •January 14-15, 2011 SCA Conference •6 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 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 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 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 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 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 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 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 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 Case # 5 : what will you do ? 1. Atropine 2. Isuprel drip 3. Epinephrine drip 4. External pacing 5. TiiTemporary pacing wire 6. Implant transvenous permanent pacemaker Follow Up • Implanted dual chamber pacemaker •January 14-15, 2011 SCA Conference •16 Case # 6 • 8dayold8 day old • 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 Case # 6: What will you do ? 1. Beta blocker 2. Amiodarone 3. Surgical resection 4. AICD 5. CdiTCardiac Transpl ant •January 14-15, 2011 SCA Conference •18 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 dis tress • CT guided Bx confirmed rhabdomyoma • Heart Tx at age 6 months Syncope - History • Most important aspect of syncope evaluation – Ot?SddOnset? Sudden sugges ts arr hthihythmia – Prodrome? If yes, suggests neurocardiogenic – Positional? Yes, then orthostatic – Supine? More concerning for arrhythmias – Preceding events? Swallowing/cold water/urination s uggest neu rocardiogenic – Witnesses? May provide best history •January 14-15, 2011 SCA Conference •19 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 •Bruggpada pattern • ARVD pattern • Important co-morbidities – Severe anemia – Electrolyte disturbances •January 14-15, 2011 SCA Conference •20.