 Understand the term syncope.  Differentiate the serious causes of syncope from those that are benign.  Know the appropriate testing needed in the evaluation of syncope based upon the presenting history.

 Palpitations - sensation of strong, rapid, or irregular heart beats.  Syncope – transient loss of consciousness and postural tone due to generalized cerebral ischemia with rapid and spontaneous recovery.  Presyncope - no complete loss of consciousness occurs.

 Disorders without impairment of consciousness: Drop attacks. Cataplexy. Psychogenic pseudo-syncope. Transient ischemic attacks.  Disorders with loss of consciousness: Metabolic disorders. Epilepsy. Intoxications. Vertebrobasilar transient ischemic attacks.

 Affects 15% of children between 8-18 Y  Uncommon under age 7 Y therefore think about:  Seizure disorders.  Breath holding.  Primary cardiac dysrhythmias.  Cardiovascular causes unusual but life- threatening  Congenital malformations.  Valvular disease.  Electrical abnormalities.

ObservationSeizure Inadequate Perfusion OnsetSudden More gradual DurationMinutesSeconds JerksFrequentRare Headache Frequent (after) Occasional (before) Confusion after FrequentRare IncontinenceFrequentRare Eye deviation Horizontal Vertical (or none) Tongue biting FrequentRare ProdromeAuraDizziness EEG Often abnormal Usually normal

Orthostatic Cardiac Arrhythmia Structural Cardio- Pulmonary 1 Vasovagal Carotid Sinus Situational  Cough  Post- Micturition 2 Drug-Induced Autonomic Nervous System Failure  Primary  Secondary 3 Brady  SN Dysfunction  AV Block Tachy  VT  SVT Long QT Syndrome 4 Acute Myocardial Ischemia Aortic Stenosis HCM Pulmonary Hypertension Aortic Dissection Neurally- Mediated Unexplained Causes = Approximately 1/3

 Vasovagal.  Situational.  Psychiatric.  Long QT.  WPW syndrome.  RV dysplasia.  Hypertrophic cardiomyopathy.  Catecholaminergic VT.  Other genetic syndromes.

 Vasovagal Events:  30% to 50% of cases.  Decreased PVR.  Decreased venous return.  Decreased cardiac output.  Hypotension.  Bradycardia.  In teens – think about pregnancy and drug abuse.

 Is the loss of consciousness attributable to syncope or not?  Is heart disease present or absent?  Are there important clinical features in the history that suggest the diagnosis?

Questions about circumstances just prior to attack  Position (supine, sitting, standing)  Activity (rest, change in posture, during or immediately after exercise, during or immediately after urination, defecation or swallowing)  Predisposing factors (crowded or warm place, prolonged standing post-prandial period) and of precipitating events (fear, intense pain, neck movements) Questions about onset of the attack  Nausea, vomiting, feeling cold, sweating, pain in chest

Questions about attack (eye witness):  Skin color (pallor, cyanotic).  Duration of loss of consciousness.  Movements ( tonic-clonic, etc.).  Tongue biting. Questions about the end of the attack:  Nausea, vomiting, diaphoresis, feeling cold, muscle aches, confusion, skin color, wounds.

Questions about background:  Number and duration of syncope spells.  Family history of arrhythmic disease or sudden death.  Presence of cardiac disease.  Neurological disease.  Medications (Hypotensive, negative. chronotropic and antidepressant agents).

Neurally-Mediated Syncope:  Absence of cardiac disease.  Long history of syncope.  After sudden unexpected, unpleasant sensation.  Prolonged standing in crowded, hot places.  Nausea vomiting associated with syncope  During or after a meal.  With head rotation or pressure on carotid sinus.  After exertion.

Syncope due to orthostatic hypotension:  After standing up.  Temporal relationship to taking a medication that can cause hypotension.  Prolonged standing.  Presence of autonomic neuropathy.  After exertion.

Cardiac Syncope:  Presence of structural heart disease.  With exertion or supine.  Preceded by palpitations.  Family history of sudden death.

 Vital signs:  Heart rate.  Orthostatic blood pressure change.  Cardiovascular exam: Is heart disease present?  ECG: Long QT, pre-excitation, conduction system disease.  Echo: LV function, valve status, HCM.  Neurological exam.

 Classically, abnormal if systolic BP decreases by more than 20 points and/or pulse. increases in pulse rate of more than 20 beats per minute after a change from supine to standing.  If there is only a pulse increase but no drop in blood pressure, the test is less significant.

 Distinguish true syncope from syncope mimics.  Determine presence of heart disease and risk for sudden death.  Establish the cause of syncope with sufficient certainty to:  Assess prognosis confidently.  Initiate effective preventive treatment.

 Survival with and without syncope (adults and children).  6-month mortality rate of greater than 10%.  Cardiac syncope doubled the risk of death.  Includes cardiac arrhythmias. No Syncope Vasovagal/other Cardiac Cause Follow-Up (yr) Probability of Survival Soteriades ES, et al. N Engl J Med. 2002;347:878.

 Yield for specific diagnosis low (5%).  Risk free and relatively inexpensive.  Abnormalities (BBB, previous MI, nonsustained VT) guide further evaluation.  Recommended in almost all patients.

 Routine use not recommended  May be glucose?  Should be done only if specifically suggested by H&P.

 EEG - not useful unless seizures.  Brain imaging - not useful unless focality.  Neurovascular studies.  No studies.  May be useful if bruits, or hx suggests vertebrobasilar insufficiency.

 History is key!!!!  Orthostatics:  take the time to do them correctly.  Cardiac vs Non-cardiac:  If you are not confident that it is NOT cardiac  REFER.  ECG  Use it if you got ‘em!

 11-year-old girl passed out during reading; awoke after 3 min.  She was stiff with eyes rolled back ~ approx. 3 min.  Now awake and alert; no retractions; skin color is normal.

 Normal appearance, normal breathing, normal circulation.  Vital signs: HR 70; RR 20; BP 90/60; T 37.7  C Wt 39 kg; O 2 sat 99%.  Three similar episodes; Preceded by palpitations,one of them associated with “exercise.”  PMH and FH: Negative.

 What is your general impression of this patient?

 Loss of consciousness.  Lasted only a few minutes.  Minimal or no postictal state.  No stigmata of seizure: Urinary incontinence, bitten tongue, witnessed tonic-clonic activity.

 Is the loss of consciousness attributable to syncope or not?  Is heart disease present or absent?  Are there important clinical features in the history that suggest the diagnosis?

 Stable  Patient with syncope.  In no distress; normal exam.  Concerning/ominous history. What are your initial management priorities?

 Laboratory is often normal but may include:  Electrolytes / Ca ++, Mg ++, PO 4.  CBC with differential.  cardiac enzyme.  Radiology:  CXR offers little.  CT or MRI of the brain and neck may be indicated if considering seizures or injury

 ECG/Holter.  Echocardiography  Cardiac MRI  Continuous cardiac monitoring  EEG  Genetic testing  Stress ECG

Differntial diagnosis :  Structural heart defect : Known Congenital heart disease (Ebstein’s anomaly,LTGA,ASD) Hypertrophic cardiomyopathy Anomalous origin of the LCA Myocarditis Arrhythmogenic RV dysplasia Coronary artery disease Primary or secondary pulmonary hypertension.

 Normal heart structure.  WPW syndrome.  Long or short QT syndrome.  Brugada syndrome.  CPVT.

QT (corrected) QTc= QT (msec) √R-R (sec) = 640/ 1.05 = 610 msec > 450 m sec is long Long QT syndrome (Jervell-Nielson-Lange)

 Inherited genetic disorder that puts the child at risk for paroxysmal ventricular tachcardia /ventricular fibrillation and sudden death.  May also result from electrolyte imbalance, malnutrition (anorexia and bulimia), myocarditis and CNS trauma  Speculation that it may be associated with SIDS (unproven)  No warning; results in death.

 Long QT syndrome:  Congenital long QT associated with hypertrophic cardiomyopathy.  Long QT defined as corrected QT longer than 0.44 s  T wave alternans sometimes present.  Can have normal ECG in the department.  Two clinical syndromes not associated with structural heart disease: Romano-Ward and Jervell-Lange- Nielsen.