1 Pediatric syncope it is not just vasovagalDr Neeraj Aggarwal Pediatric Cardiologist Department of Pediatric Cardiac sciences Sir Ganga Ram Hospital

2 Case A 8 years old girl is brought to OPD for evaluation of a fainting episode during morning school assembly She describes feeling very warm, nauseated and light-headed The loss of consciousness lasted for 20 to 30 seconds. Witnessed describe her as extremely pale before passing out Past history of similar episodes during assembly and she now knows the prodromal symptoms

3 Causes of syncope Commonly --Neurally mediated syncope(vasovagal)Cardiovascular syncope Primary/structural heart diseases Obstructive lesions- Aortic stenosis ,Pulmonary Stenosis Pulmonary hypertension/Eisenmenger syndrome Hypertrophic Cardiomyopathy Arrhythmias Tachyarrhythmias-Long QT syndrome,Brugada syndrome (familial ventricular fibrillation),Supraventricular tachycardia,Ventricular tachycardia Bradyarrhythmias -Sick sinus syndrome,Heart block

4 Diagnostic aims Distinguish true syncope from syncope mimicsDetermine site of origin –vasovagal or cardiac

5 Syncope Mimics Seizures Sleep disordersSomatization disorder (psychogenic) Acute intoxication (e.g., alcohol) Trauma/concussion Hypoglycemia Hyperventilation Munchausen syndrome by proxy ( factitious disorder)

6 Diagnostic Inventory Initial Examination-must in allDetailed patient history and exam Supine and upright blood pressure ECG Additional Cardiac work up Echocardiography -Holter Event Recorder Insert-able Loop Recorder (ILR) Special Investigations Head-up tilt test (HUTT) Electrophysiology study 24 hr blood pressure monitoring

7 Diagnostic Assessment: Yields

8 Common Causes of SyncopeNeurally mediated - vaso vagal syncope , situational –church ,rock concert, post micturition ,cough Orthostatic hypotension-drug induced,Autonomic dysfunction Cardiac arrhythmias –long QT syndrome, brady or tachy arrhythmias Structural heart diseases – PAH ,Aortic stenosis ,Hypertrophic cardiomyopathy

9 Detailed history Position (supine, sitting, or standing)Activity: rest, change in posture,, during or immediately after urination, defecation, cough or swallowing,during or after exercise

10 Relation to exercise History ImplicationsDuring exercise or with exertion Arrhythmias ,PAH,HCM,AS During swimming/loud noise LQTS until proved otherwise Prolonged motionless standing Vaso-vagal Vaso vagal syncope that is associated with exercise does exist, but a more serious cardiac cause should always be eliminated in exercise-related syncope For exercise-induced syncope, an Exercise Stress Test is mandatory to look for ST-T wave changes (coronary insufficiency and catecholamine- sensitive dysrhythmias)

11 History Predisposing factors (e.g. crowded or warm places, prolonged standing, postprandial period) 4. Precipitating events (e.g. fear, intense pain, position)

12 Triggers Of vaso- vagal syncope In The YoungEmotional circumstances and pain, such as venipunctures, immunizations ,blood sight Prolonged motionless standing, especially in combination with warm temperature, confined spaces, crowded rooms (‘church syncope,school assembly ’) Fasting, lack of sleep, fatigue, menstruation, illness with fever Micturition ,hair cutting

13 Remember :Trigger may change in a patient from time to timePost exercise (i.e., after termination of long runs or vigorous bursts of activity during competitive sports) Hyperventilation and straining (self-induced syncope) Stretching -- shaving with hyperextended neck Standing up quickly or arising from squatting

14 Other history History ImplicationsFamily history of early sudden death, congenital arrhythmogenic heart disease. LQTS, Familial Cardiomyopathy Surgery for congenital heart disease Arrhythmias Sensorineural deafness LQTS Associated with medications (antihypertensive, antidepressant agent, antiarrhythmic, diuretics, and QT prolonging agents) LQTS prolonging drugs,hypotension,hypoglycemia)

15 Red flag signs Chest pain, dyspnoea, palpitationsHistory of heart disease/cardiac surgery Syncope during exercise, with swimming, with loud noise or in sleep is typical of long QT syndromes. Family history of deafness. Family history of sudden unexpected death (young age ,previous sib) Prolonged loss of consciousness > 5 min Severe headache, focal neurological deficits, diplopia, ataxia, or dysarthria before the syncope

16 Physical Examination Goal - To check for orthostatic hypotension To rule out a significant heart lesion

17 Detailed examination with special attention to CVS and CNSVital signs in supine and then after standing for 3 to 5 minutes Palpate: Displaced apex - lift/heave RV lift/heave Thrills Palpable S2 Murmurs of outflow obstruction-usually loud Loud P2 indicative of pulm HTN ? soft sign

18 Orthostatic hypotensionFall in systolic of 20 mm Hg or of 10 mm Hg in diastolic (within 3 minutes of standing from the sitting or supine position) Postural tachycardia syndrome- Supine to standing –HR increase of >35 beats/min , within 10 minutes of standing

19 Investigations ECG

20 WPW

21 WPW

22 CHB

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24 Long QTc -Bazett’s Correction= QT/√R-R intervalFemale => 460 ms Male => ms (95th percentile values) >470 and >480 (99th percentile) almost prove LQTS in absence of secondary causes

25 Stepwise approach to correct measurement of the QT intervalUse lead II. Use lead V5 alternatively if lead II cannot be read. Draw a line through the baseline (preferably the PR segment)

26 If the T wave is broad, the tangent is drawn

27 If the T wave has two positive deflections, the taller deflection should be chosen

28 If the T wave is biphasic, the end of the taller deflection should be chosen

29 The QT interval starts at the beginning of the QRS interval and ends where the tangent and baseline cross

30 We have diagnosed long QTc- will genetic tests helpManagement will change Prediction of recurrence in next child

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32 Further Evaluation or Not?If history is typical, exam and ECG are normal, then further testing &/or referral not necessary (eye witness accounts) Explanation Reassurance No risk of Sudden death, benign & temporary Start simple therapy –postural ,avoid holding up toddlers Video recording the event

33 Other Diagnostic TestsEchocardiogram Head-Up Tilt (HUTT) Test Includes drug provocation (NTG, isoproterenol) Ambulatory ECG Holter monitoring Event recorder Intermittent vs. Loop Insertable Loop Recorder (ILR) Electrophysiology Study (EPS)

34 Other Diagnostic TestsIf history is not typical for vasovagal syncope Any of the red flag signs present

35 Case A 3 yr old child ,having syncopal event while climbing and having early tiredness Examination shows loud 3/6 ejection systolic murmur in left 2 ICS ECG –Left ventricular hypertrophy Next step –ECHO must

36 left ventricular hypertrophy: R wave in V6 = 32mm

37 case ECHO –severe valvular aortic stenosis, bicuspid aortic valveUnderwent Balloon aortic valvotomy

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39 Holter Monitoring Cases in which an arrhythmia is suspectedto eliminate frequent ectopy, VT, SVT, bradycardia, intermittent WPW, Heart block or pauses

40 Endless-loop recorders – event recordersUsed to capture and save episodes even minutes after they have occurred Time interval recorded before the button is pushed is often programmed

41 Implanted loop recordersIf difficult to capture an episode with an external loop recorder or if the episodes are quite far apart Automatically or can be triggered by the patient to save an event

42 Role of Head up tilt table testSensitivity low – % Specificity high % So valuable to prove a vasovagal syncope (which can be done by a basic history ,exam and ECG) To rule out a life threatening illness –ECHO more useful

43 Summary Chest pain, dyspnoea, palpitations ,history of heart diseaseA careful history and examination including blood pressure and heart rate measured lying and standing, along with ECG is generally the only evaluation required Presence of Red flag signs – needs a cardiac work up Chest pain, dyspnoea, palpitations ,history of heart disease Syncope during exercise, with swimming, with loud noise or in sleep is typical of long QT syndromes. Family history of deafness/sudden unexpected death Prolonged loss of consciousness > 5 min

44 Thanks Dr Neeraj Aggarwal Pediatric Cardiologist Sir Ganga Ram Hospital