Syncope in Children DR HUDA I KHALEEL CONSULTANT PEDIATRIC CARDIOLOGY KSMC

Objectives 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.

Presyncope - no complete loss of consciousness occurs.

Introduction Syncope is a sudden, transient loss of consciousness associated with loss of postural tone and subsequent spontaneous, complete recovery. Although the symptoms of syncope can appear concerning, most patients are not at risk of significant morbidity or mortality. it is most often benign,

Syncope in children 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.

Syncope Mimics 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.

Differential Diagnosis of Syncope: Seizures vs Hypotension Observation Seizure Inadequate Perfusion Onset Sudden More gradual Duration Minutes Seconds Jerks Frequent Rare Headache Frequent (after) Occasional (before) Confusion after Incontinence Eye deviation Horizontal Vertical (or none) Tongue biting Prodrome Aura Dizziness EEG Often abnormal Usually normal

Unexplained Causes = Approximately 1/3 Causes of True Syncope Neurally- Mediated 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 Unexplained Causes = Approximately 1/3

Likely Causes In Children Vasovagal. Situational. Psychiatric. Long QT. WPW syndrome. RV dysplasia. Hypertrophic cardiomyopathy. Catecholaminergic VT. Other genetic syndromes.

Syncope in children 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.

Syncope: Key questions to address with initial evaluation 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?

Syncope: Important Historical Features 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

Syncope: Important Historical Features 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.

Syncope: Important Historical Feature 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).

Clinical Features Suggesting Specific Cause of Syncope 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.

Clinical Features Suggesting Specific Cause of Syncope 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.

Clinical Features Suggesting Cause of Syncope Cardiac Syncope: Presence of structural heart disease. With exertion or supine. Preceded by palpitation. Family history of sudden death.

Initial Exam: COMPLETE Physical EXAMINATION 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. HCM—Hypertrophic Cardiomyopathy Brignole M, Alboni P, Benditt D, et al. Guidelines on management (diagnosis and treatment) of syncope—Update 2004. Europace. 2004;6:467-537.

Orthostatic Measurements 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.

Diagnostic Objectives 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.

Electrocardiogram 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.

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

Neurologic Testing 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.

Cardiac vs Non-cardiac: -Is it Syncope?- 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

Case study 1 11-year-old girl passed out during reading; after 3 min. She was stiff with eyes rolled back ~ approx. 3 min. Now awake and alert; no retractions; skin color is normal.

Case study 1 Normal appearance, normal breathing, normal circulation. Vital signs: HR 70; RR 20; BP 90/60; T 37.7 C Wt 39 kg; O2 sat 99%. Three similar episodes; Preceded by palpitations ,one of them associated with “exercise.” PMH and FH: Negative.

Case study 1 What is your general impression of this patient?

Clinical Features: Your First Clue 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.

Syncope: Key questions to address with initial evaluation 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?

Case study 1 Stable Patient with syncope. In no distress; normal exam. Concerning/ominous history. What are your initial management priorities?

cardiac enzyme. Diagnostic Studies Electrolytes / Ca++, Mg++, PO4. Laboratory is often normal but may include: Electrolytes / Ca++, Mg++, PO4. 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

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

Case study 1 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.

Long or short QT syndrome. Brugada syndrome. Case study 1 Normal heart structure. WPW syndrome. Long or short QT syndrome. Brugada syndrome.

Case Study 5

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

Speculation that it may be associated with SIDS (unproven) Long QT Syndrome 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.