Syncope in Children & Adolescents Red Flags

1. Syncope in Children & AdolescentsRed Flags Ramallah 2011 Dr. Ayob Hamdan, Pediatrician Consultant

3. INTRODUCTIONWhat is syncope? • Syncope is a sudden, brief loss of consciousness; loss of postural tone & recovery is spontaneous. • Up to 15 percent of children experience a syncopal episode prior to the end of adolescence.

4. Recoveryis usually immediate, with occasional brief period of confusion. • Preceding symptoms: lightheadedness, nausea, visual disturbances and palpitation.

5. Etiology • The etiology in children is most often benign. • Syncope can also occur as the result of more serious (usually cardiac) disease with the potential of sudden death.

6. The usual complaints Fainted.. Standing long.. Following exercise.. Hot weather.. Light-headed.. Seeing black.. Seeing stars.. Nausea.. Abdominal cramps.. Racing heart.. Rapid standing from sitting down.. No breakfast.. Seeing blood.. Blood draws.. Urination.. Turning head rapidly.. Deep emotions.. Forgot what happened.. Headache.. Less common: Palpitation.. Brief convulsions.. Brief confusion

7. Mechanism of the common syncope baseline Pre-syncope Syncope Recovery BP HR

8. COMMON CONDITIONS • Vasovagal syncope (neurocardiogenic) is the most common cause of syncope among children ~50%. • Breath holding spells;typically occur in children between 6 months and 24 months old; 2 types pallid and cyanotic. • Orthostatic hypotension . • Toxic exposure (opiates, alcohol, carbon monoxide ). • Medications (barbiturates, tricyclic antidepressants, and phenothiazines)

11. OTHER CONDITIONS • Hypoglycemia (Type 1 DM) • Supraventricular tachycardia (Rare). • Bradycardia(VR)corrective surgery for congenital heart disease, hypervagotonia, hypothyroidism, and medications.

12. CONDITIONS THAT MIMIC SYNCOPE • Seizures — a seizure typically includes loss of consciousness and postural tone. • Migraine syndromes (Basilar migraine) • Hysteria/conversion disorder. • Hyperventilation. • Choking game.

13. All Children with Syncope Benign Syncope 98% + Children with Serious Conditions Diagnosis and Appropriate Management Sudden Death without Diagnosis

14. Red flags in history • During exercise (not following exercise) • Preceding chest pain • Resuscitation required (CPR) • Family history of sudden death • Deafness • Progressive symptoms: more frequent fainting, more prolonged episodes. • Preceding palpitation.

15. Cardiac Causes of Fainting • Electrical • Structural

16. Electrical Disturbances Less common to cause syncope than structural. May be inherited.

17. Long Q-T Syndrome • Repolarization Disorder Which causes polymorphic ventricular tachycardia and sudden death. • QT is long (>0.45 seconds). • May be acquired or congenital. • Acquired: medications induced or electrolyte disturbance . • Congenital: Romano-Ward syndrome and Jervell Lange-Nielsen syndrome .

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

22. Brugada syndrome Patients with Burgada syndrome have Characteristic patterns on ECG. Increased risk of sudden death .

25. Catecholaminergic polymorphic ventricular tachycardia • CPVT cause ventricular tachycardia and ventricular fibrillation. • Triggered by emotional or physical stress. • Familial and sporadic cases may occur.

26. Preexcitation syndrome • Wolff-Parkinson-White which cause supraventricular tachycardia. • The incidence of WPW syndrome is between 0.1% and 0.3% of the general population.

27. Congenital short QT syndrome

28. 14 year old with short episodes of palpitation WPW syndrome

29. Congenital short QT syndrome • QT < 0.3 sec • Cause atrial fibrillation syncope or sudden cardiac death. • 3 Genetic mutations described .

30. Structural heart disease  • Hypertrophic cardiomyopathy . • Coronary artery anomalies . • Arrhythmogenic right ventricular cardiomyopathy  • Valvar aortic stenosis . • Dilated cardiomyopathy. • Pulmonary hypertension. • Acute myocarditis .

31. Exampels

32. Coronary artery from the wrong aortic sinus Left coronary from the right coronary sinus, inter-arterial course Right coronary from the left coronary sinus, inter-arterial course

33. Examples of coronary anomalies Right coronary artery from pulmonary artery, four-year-old

34. 4 year old with recurrent faints Only 4 years old (young for the typical benign syncope) Occasional convulsions with the fainting episodes Examination was normal except for right ventricular heave ECG..

35. RV hypertrophy, right atrial hypertrophy

36. Echocardiography was diagnostic; pulmonary hypertension No etiology found  primary PHTN Pulmonary vasodilators started, only transient response Died suddenly 2 years after the diagnosis

37. 9-year-old with LOC while playing basketball at school, has systolic murmur Left ventricular hypertrophy due to aortic stenosis

38. 13 year old boy with palpitation and family history of early deaths LVH, PVC, Hypertrophic cardiomyopathy

39. Children with congenital heart disease, including those who have undergone surgical palliation or correction, are also at risk ofmalignant arrhythmias. sudden cardiac death .

40. Fainting during exercise • May indicate: • Cardiac conditions where there is obstruction to blood flow when demand is high • Valvar stenosis • coarctation of aorta • obstructive cardiomyopathy • Congenital coronary abnormalities • Anomalous origin of coronary arteries • Anomalous course of coronary arteries • Exercise induced arrhythmias • Long QT • Arrhythmogenic right ventricular dysplasia (rare) • Brugada syndrome (rare)

41. Palpitation prior to fainting • May indicate: • Episodes (paroxysms) of supraventricular tachycardia • e.g. Wolffe-Parkinson-White syndrome • Other conditions • Long QT syndrome • Atrial fibrillation • Remember: Palpitation may occur in common faint

42. Family history of sudden death • Long QT syndrome (ask about deafness) • Hypertrophic cardiomyopathy • Arrhythmogenic right ventricular dysplasia (V rare)

43. Red flags in Physical exam Bradycardia, irregular rhythm Heart block, atrial fibrillation, sinus node dysfunction Hypertension, radio-femoral delay coarctation of aorta Systolic murmur of aortic stenosis Systolic murmur of hypertrophic cardiomyopathy (louder in standing, valsalva) Distant heart sounds, pulsus paradoxus pericardial effusion Right ventricular heave pulmonary hypertension

44. What to look for in an ECG? Bradycardia, irregular rhythm Heart block, atrial fibrillation, sinus node dysfunction Long QT syndrome Wolffe-Parkinson-White syndrome Findings supportive of structural heart disease: left ventricular hypertrophy (aortic stenosis, coarctation of aorta, hypertrophic cardiomyopathy) Right atrial and Right ventricular hypertrophy (pulmonary hypertension) left atrial dilatation, RV hypertrophy (mitral stenosis) ST segment changes (pericardial effusion, coronary anomalies)

45. Summary.. A child with syncope

46. Build an algorithm Child with syncope Detailed history Red flag Benign history PE + ECG+Echo Physical examination ECG+Echo Red flag Normal examination Refer to cardiologist Diagnosis ECG Normal ECG Progressive symptoms, refractory fainting Reassure and follow patient as common faint

47. PSP 7th Medical Conference Thank you For your attention! I hope you found this discussion helpful and informative!