Sidra Medical & Research Center Syncope in Children May, 2017 Jai Udassi, MD, FAAP, FACC, FAHA Associate Professor of Pediatrics Director Pediatric Cardiac ICU Chief Critical Care (A) Sidra Medical & Research Center

Disclosure I have no financial or any other conflict

Goals/Objectives Causes of syncope in pediatric patients Understand the elements in the history or physical examination that may be related to a life-threatening cause Describe the basic evaluation for a patient who presents with syncope Screen and identify patients who require referral to a pediatric subspecialist

Definition Syncope is a sudden and transient loss of consciousness and postural muscle tone that reverses without intervention.

Epidemiology Approximately 15% to 25% of children and adolescents experience at least 1 episode of syncope before adulthood The incidence peaks in the late teenage years and occurs more commonly in females Syncope accounts for as many as 3% of all emergency department visits in the pediatric population Although a substantial portion of syncope in the adult population is due to cardiac causes, most syncope in the pediatric population is benign

Case # 1 A 14-year-old girl with no significant past medical history presents to the clinic/ER following a syncopal episode. She reports that she had been standing in church and felt lightheaded before passing out. The event was witnessed and her parents describe brief period they noticed that she was trying to hold on to something, that is when she passed out. She woke up 2 seconds after she passed out and was alert and oriented. What is the most likely cause of the syncope? What further evaluation is necessary? What recommendations would you make for this patient? Would you refer her to a pediatric cardiologist or neurologist?

Case # 2 A 10-year-old boy with no significant past medical history presents to the clinic/ER following a syncopal episode. He explains that he was running a 100-meter dash and passed out in the middle of running. He had no symptoms before syncope. He woke up after 4 to 5 seconds but was very confused and did not recognize his track coach or teammates. What are the potential causes of the syncope? What further evaluation is necessary? What recommendations would you make for this patient? Would you refer him to a pediatric cardiologist or neurologist?

Case # 3 A 14-year-old girl with no significant past medical history presents to the clinic/ER following a syncopal episode. She explains that she was playing football and passed out in the middle of running. She had a sharp chest pain just before her syncope. She woke up after 10 seconds but was very confused and felt week. What are the potential causes of the syncope? What further evaluation is necessary? What recommendations would you make for this patient?Would you refer her to a pediatric cardiologist or neurologist?

Differential Diagnosis / Causes 75% of Pediatric Syncope Pediatrics in Review, Vol. 37 No. 4 April 2016

Vasovagal Vasovagal syncope is also referred to as neurocardiogenic syncope, vasodepressor syncope, or simple/common faint Typically, a prodrome lasts a few seconds to 1 minute and is followed by syncope that usually lasts less than 1 minute Upright posture results in venous pooling in the lower extremities C-fiber/mechanoreceptor activation results in a reflexive increase in parasympathetic tone (decreased heart rate and blood pressure) with the systolic blood pressure decreasing up to 40 to 80 mmHg Bradycardia may progress to asystole that lasts 3 to 40 seconds in 4% to 6% of patients

Vasovagal-Prodrome Typical symptoms of presyncope are dizziness, vision changes, a warm feeling, nausea, or an irregular heartbeat before complete loss of consciousness, Although on rare occasions there are no presyncopal warning signs. Some patients frequently have isolated episodes of presyncope without subsequent loss of consciousness

Postural Orthostatic Tachycardia Syndrome (POTS) POTS is a condition in which the heart rate increases more than 30 beats per minute, without hypotension (inappropriate sinus tachycardia) within 10 minutes of assuming an upright position. Symptoms can be syncopal or presyncopal POTS has been associated with chronic fatigue, gastrointestinal problems, headaches, poor sleep, difficulty concentrating, and decreased exercise tolerance. In some patients, there is a substantial psychological influence to the symptoms. POTS has a female-to-male ratio of 5:1. Many affected patients do not have true syncope but may have marked debilitation from presyncopal symptoms. Dr. Cannon-April 2016

Cardiac Syncope Pediatrics in Review, Vol. 37 No. 4 April 2016

WPW Syndrome-Delta wave

Prolong QTc

Prolong QTc

Calculate (corrected QT) QTc The most commonly used equation is Bazett’s formula (the QT interval in seconds divided by the square root of the previous QRS-to-QRS interval in seconds). The upper limit of normal for a corrected QT interval is 440 milliseconds in males and 450 milliseconds in females. Bazett’s formula: QTC = QT / √ RR

Prolong QTc (Pearl)

Torsades de pointes Multiform V Tach with progressive changes in amplitude of QRS complexes separated by narrow transition QRS Polarity of QRS repetitively twists around an iso-electric baseline Can progress to V Fib

Brugada Syndrome V1 pattern in BrS Normal V1 Brugada syndrome (BrS) is;genetic disease that is characterised by abnormal electrocardiogram (ECG), It is a Sodium channel problem

V1 to V3, (Normal VS. BrS)

V Tach

LCAPA (left coronary artery from Pulmonary artery)

Diagram shows the spectrum of pathophysiologic changes that take place after birth in patients with ALCAPA syndrome Angiogram with LCAPA Pena, E. et al. Radiographics 2009;29:553-565

Second-Degree Heart Block Mobitz Type II Constant PR interval before a skipped ventricular conduction

Third-Degree Heart Block Complete Complete dissociation of atrial and ventricular conduction P wave and PR interval normal Junctional pacemaker – narrow QRS Ventricular pacemaker – wide QRS Rate 30 – 50 beats/min

Approach to a Syncope in Children History, Family History Physical Examination EKG Blood work, CBC, Electrolytes Pregnancy Test CT/MRI head EEG Tilt table Event Monitor Holter Pediatrics in Review, Vol. 37 No. 4 April 2016

Case # 1 A 14-year-old girl with no significant past medical history presents to the clinic following a syncopal episode. She reports that she had been standing in church and felt lightheaded before passing out. The event was witnessed and her parents describe brief period they noticed that she was trying to hold on to something, that is when she passed out. She woke up 2 seconds after she passed out and was alert and oriented. What is the most likely cause of the syncope? What further evaluation is necessary? What recommendations would you make for this patient? Would you refer her to a pediatric cardiologist or neurologist?

Case # 1 has Vasovagal Syncope A 14-year-old girl with no significant past medical history presents to the clinic following a syncopal episode. She reports that she had been standing in church and felt lightheaded before passing out. The event was witnessed and her parents describe brief period they noticed that she was trying to hold on to something, that is when she passed out. She woke up 2 seconds after she passed out and was alert and oriented. What is the most likely cause of the syncope? What further evaluation is necessary? What recommendations would you make for this patient? Would you refer her to a pediatric cardiologist or neurologist?

Case # 2 A 10-year-old boy with no significant past medical history presents to the clinic following a syncopal episode. He explains that he was running a 100-meter dash and passed out in the middle of running. He had no symptoms before syncope. He woke up after 4 to 5 seconds but was very confused and did not recognize his track coach or teammates. What are the potential causes of the syncope? What further evaluation is necessary? What recommendations would you make for this patient? Would you refer him to a pediatric cardiologist or neurologist?

Case # 2 EKG

Case # 2 has Prolong QT Syndrome A 10-year-old boy with no significant past medical history presents to the clinic following a syncopal episode. He explains that he was running a 100-meter dash and passed out in the middle of running. He had no symptoms before syncope. He woke up after 4 to 5 seconds but was very confused and did not recognize his track coach or teammates. What are the potential causes of the syncope? What further evaluation is necessary? What recommendations would you make for this patient? Would you refer him to a pediatric cardiologist or neurologist?

Case # 3 A 14-year-old girl with no significant past medical history presents to the clinic following a syncopal episode. She explains that she was playing football and passed out in the middle of running. She had a sharp chest pain before her syncope. She woke up after 10 seconds but was very confused and felt week. What are the potential causes of the syncope? What further evaluation is necessary? What recommendations would you make for this patient?Would you refer her to a pediatric cardiologist or neurologist?

Case # 3 EKG

Case # 3 has ALCAPA A 14-year-old girl with no significant past medical history presents to the clinic following a syncopal episode. She explains that she was playing football and passed out in the middle of running. She had a sharp chest pain before her syncope. She woke up after 10 seconds but was very confused and felt week. What are the potential causes of the syncope? What further evaluation is necessary? What recommendations would you make for this patient?Would you refer her to a pediatric cardiologist or neurologist?

Summary Most causes of syncope can be determined by a thorough detailed history, including family history, it will take time! Syncope is a typically benign entity in young patients and usually has a good prognosis Syncope during active exercise, syncope that occurs without warning, and syncope preceded by severe chest pain or rapid palpitations possibly have cardiac origins The primary therapies for reflex syncope (vasovagal) are conservative measures such as increased fluid and salt intake and a routine exercise program Medications are not particularly effective at preventing recurrent episodes of syncope, with the exception of midodrine, which may be beneficial in children with a prominent hypotensive response