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Cardiac Causes of Pediatric Syncope:

Cardiac Causes of Pediatric Syncope:. Finding the Needle in the Haystack. Eric Johnson, MD April 18, 2019. Disclosures. None. Objectives. List the most common causes of syncope in pediatric patients Describe the mechanism of vasovagal syncope in one sentence

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Cardiac Causes of Pediatric Syncope:

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  1. Cardiac Causes of Pediatric Syncope: Finding the Needle in the Haystack Eric Johnson, MD April 18, 2019

  2. Disclosures • None

  3. Objectives • List the most common causes of syncope in pediatric patients • Describe the mechanism of vasovagal syncope in one sentence • Know what to do if you suspect an arrhythmia (such as SVT) • Identify features that may be related to a life-threatening cardiac cause

  4. Outline • Cases • Definition • Epidemiology • Causes • Dysautonomia • Cardiac • Clinical evaluation • When to consider referral

  5. Case #1 • 13 y.o female • Unremarkable medical history • Just finished tryouts for volleyball, was standing in a circle during announcement of who made the varsity team • Felt dizzy and lightheaded, rapid palpitations, and “slumped to the floor,” losing consciousness for 1 minute • Afterward, she felt weak and had shaky hands for about 15 minutes

  6. Case #2 • 5 y.o. male • At age 2, he was running during recess, collapsed, neck extended, eyes “rolled back,” appeared to stop breathing, had fine movements of shoulders and arms, and turned “purple” • Unconscious for 1 minute, seemed confused for a few seconds, then back to baseline • Since then, similar episodes about once a year

  7. Definition • A sudden and transient loss of consciousness and postural tone that reverses without intervention.

  8. Epidemiology • 15 – 25% experience syncope before adulthood • Disproportionately affects teenage females • 3% of all pediatric ED visits • Most cases are benign • Rarely, syncope is a harbinger of a life-threatening condition, usually cardiac

  9. Pathophysiology • Critical cerebral blood flow = 60 mL/min/100 g • Cerebral perfusion is usually maintained by autoregulation • When autoregulation fails to overcome a sudden drop in systemic arterial blood pressure, pre-syncope or syncope occurs • In benign cases, this reconstitutes adequate cerebral blood flow

  10. Causes • Syncope • Cardiac • Dysautonomia • Neurologic • Other • Psychogenic • Drugs/Toxins • Anemia • Hypoglycemia • Pregnancy • Breathholding • Syncope “games” • Vasovagal • Situational • POTS • Orthostatic hypotension • Structural heart disease • Arrhythmia • Myocardial dysfunction • Seizures • Migraines • Brain tumor • AV malformation • Cerebrovascular occlusive disease

  11. Vasovagal • AKA neurocardiogenic, vasodepressor, simple/common faint • Prodrome lasts a few seconds to 1 minute • Dizziness, vision changes, nausea, warmth • Syncope typically lasts less than 1 minute • Associated with changes in position

  12. Vasovagal Forceful contractions of an underfilled heart inappropriately activate C-fibers in the heart Increased parasympathetic tone and decreased sympathetic tone Bradycardia, vasodilation, and hypotension Reduction of cerebral perfusion Why did my child faint?

  13. Vasovagal • “The nerves that help regulate your child’s heart rate and blood pressure were not working properly, which led to a temporary decrease in blood flow to the brain, and subsequent loss of consciousness.”

  14. Situational • Pathophysiology and clinical presentation is similar to vasovagal • A specific trigger initiates the cascade of autonomic events leading to syncope • Sight of blood, pain, fear, hair brushing, urination

  15. POTS • Postural orthostatic tachycardia syndrome • Within 10 minutes of assuming an upright position . . . • HR increase 30-40, or > 120 beats/min • Associated with exercise intolerance, chronic fatigue, GI problems, headaches, poor sleep, difficulty concentrating, psychological problems • Female: male = 5:1

  16. Orthostatic hypotension • Within 3 minutes of assuming an upright position . . . • Decrease in systolic BP > 20 mmHg • Decrease in diastolic BP > 10 mmHg • Normal adrenergic vasoconstriction is inadequate • Associated with prolonged bed rest or standing

  17. Orthostatic vital signs • It takes 15 minutes to do it right! • Supine for 5 minutes, then BP + HR • Stand for 3 minutes, then BP + HR • Stand for 7 more minutes, then BP + HR

  18. Management of dysautonomia • Not a life threatening condition* • There is no cure, although many patients get better • Focus on improving function through: • Increase salt and water intake • Regular exercise • Avoiding triggers and caffeine • Fludrocortisone, midodrine, B-blockers, saline

  19. Causes • Syncope • Cardiac • Dysautonomia • Neurologic • Other • Psychogenic • Drugs/Toxins • Anemia • Hypoglycemia • Pregnancy • Breathholding • Syncope “games” • Vasovagal • Situational • POTS • Orthostatic hypotension • Structural heart disease • Arrhythmia • Myocardial dysfunction • Seizures • Migraines • Brain tumor • AV malformation • Cerebrovascular occlusive disease

  20. Hypertrophic cardiomyopathy • Ventricular hypertrophy without a hemodynamic cause • One of the most common inherited cardiomyopathies (autosomal dominant) • A heterogeneous group of disorders; outcomes depend on type and age • Disproportionately affects teenagers, annual mortality rate = 1%

  21. Hypertrophic cardiomyopathy • History: • Heart failure (primarily dyspnea on exertion), chest pain (both at rest or with exertion), arrhythmias (both atrial and ventricular), syncope (15-25% of patients), or sudden death • Exam: • Systolic ejection murmur (the less full the ventricle, the louder the murmur) • Systolic regurgitant murmur

  22. Coronary artery anomalies

  23. Coronary artery anomalies • Many variations • Incidence of both coming off the same sinus of Valsalva ~ 0.1 to 0.3% of births • The left coronary coming off the right sinus has higher mortality

  24. Coronary artery anomalies • History: • Exertional chest pain, syncope, or sudden death • Exam: • Unrevealing

  25. Supraventricular tachycardia (SVT) (Kim and Knight, 2017)

  26. SVT • History • Palpitations (due to an atrial arrhythmia), lightheadedness, dizziness, syncope, chest pain, sudden death • Exam • Unrevealing

  27. SVT • If a patient is having an episode of likely SVT (heart rate > 150 beats/min at rest, can be much higher): • Take blood pressure if able, consider calling pediatric cardiologist • If doing fine, get an ECG • If not, try vagal maneuvers (such as Valsalva) • Or consider local ED / 911

  28. Long QT syndrome • Disorder of ventricular repolarization • May be congenital (channelopathy) or acquired (often drug-related) • Borderline QTc = 0.44 to 0.46 msec (Sanatani et al, 2016)

  29. Long QT syndrome (Roden and Spooner, 1999)

  30. Long QT syndrome • History: • Palpitations, syncope, cardiac arrest, sudden death • Family history of congenital deafness, drownings, or accidents without precipitating factors • Exam: • Unrevealing

  31. Acute myocarditis • Inflammation of the myocardium • Pediatric patients can present with acute or fulminant disease • Most common etiology is viral infection • Coxsackie A and B, and adenovirus • Syncope may be due to ventricular dysfunction or arrhythmia

  32. Acute myocarditis • History • Ranges from subclinical to sudden death • Often with viral prodrome • Heart failure: dyspnea, exercise intolerance, gastrointestinal symptoms • Exam • Tachypnea, tachycardia, rales, gallop, regurgitant murmur, friction rub, hepatomegaly

  33. Clinical evaluation - History • Details about all of the events surrounding the episode are critical • Was it presyncopevs true syncope? • Are there risk factors for dehydration? • How long was the patient unconscious? • Family history of vasovagal episodes, cardiomyopathy, ICD, long QT, sudden death

  34. Cross sectional study of 3,445 patients (age < 18) • Presented with syncope or near-syncope to 2 EDs in Atlanta from 2009 to 2013

  35. 0.1% (n=3) were due to a previously undiagnosed cardiac etiology • 2 were SVT and 1 was myocarditis

  36. The presence of 2 out of 4 of the following historical features was 100% sensitive and 100% specific • Absence of a prodrome • Chest pain leading up to syncope • Palpitations leading up to syncope • Syncope during exercise

  37. Clinical evaluation - Exam • Distressed? • Pale? • Count a heart rate (a key skill!) • A heart rate >150 at rest may be SVT, and >220 at rest is SVT until proven otherwise • If possible, take a blood pressure • If possible listen for a heart murmur

  38. Referral is indicated for patients who: • received CPR* • were cardioverted (external or ICD)* • have findings consistent with a cardiac cause for syncope • have recurrent episodes consistent with dysautonomia • would benefit from parental reassurance

  39. Case #1 • 13 y.o. female with palpitations and syncope after volleyball • Supine HR: 58 BP: 108/50 • Standing (3 min) HR: 114 BP: 105/81 • Standing (10 min) HR: 120 BP: 77/56 • POTS

  40. Case #2 • 5 y.o. male with syncope while running

  41. Summary • Dysautonomia is the most common cause of syncope in pediatric patients • A thorough history is the key to distinguishing potentially dangerous etiologies • Practice counting high heart rates • Call us with concern for arrhythmia, or syncope with exercise/palpitations/chest pain (especially when there is no prodrome) • If a patient has known cardiovascular disease, make a written plan with family and/or provider

  42. References • Allen, H. Syncope: Avoid the Knee-Jerk Echo. AAP Gateway Journals Blog. 2016 Apr 12. • Cannon, B, and Wackel, P. Syncope. Pediatric in Review. 2016 Apr;37(4):159-68. • Hurst, D, et al. Syncope in the pediatric emergency department – can we predict cardiac disease based on history alone? J Emer Med. 2015;49(1):1-7. • Kim, S, and Knight, B. Long term risk of Wolff-Parkinson-White pattern and syndrome. Trends in Cardiovascular Medicine. Article in press. 2017 • Reybrouck, T, and Ector, H. Syncope and Assessment of Autonomic Function in Children. Moss and Adams’ Heart Disease in Infants, Children, and Adolescents, Chapter 12. Lippincott Williams & Wilkins. 2008. • Roden, D, and Spooner, P. Inherited Long QT Syndromes. Journal of Cardiovascular Electrophysiology. 1999 Dec;10(12):1664-83. • Sanatani, S, et al. Canadian Cardiovascular Society and Canadian Pediatric Cardiology Association Position Statement on the Approach to Syncope in the Pediatric Patient. J CJC. Article in press. 2016. • UpToDate, 2017. • <https://www.texaschildrens.org/departments/anomalous-aortic-origin-coronary-artery-aaoca>

  43. Thank You

  44. Misty Carlson, MD mcarlson2@peacehealth.org Eric Johnson, MD ejohnson@peacehealth.org Pediatric Cardiology(541) 222-6160 24/7/365 • Outpatient clinic consultations • Pediatric echocardiograms • Pediatric ECGs and rhythm monitors • Email or phone consultations

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