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Induced Brugada Syndrome - what are the risks?
Susan P. Torrey, MD, FAAEM, FACEP Associate Professor of Emergency Medicine Tufts University School of Medicine Baystate Medical Center, Springfield, MA
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I have no financial disclosures,
but… @STorreyMD
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Brugada Syndrome Inherited Na+ channelopathy
Autosomal dom w/ incomplete penetrance Sudden death (often during sleep) Mean age 41 +/- 15 years Men > woman (5.5 more common) Prevalence (0.02% - 2%) Asia > Europe > US
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Sudden Unexpected Death in Sleep
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Asian mythology Philippines – bangungut “to rise and moan”
Thailand – phi am “widow ghost” Japan – pokkuri “pop-off”
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Brugada syndrome, type I
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Brugada syndrome
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Brugada Syndrome Risk factors for suden death
Type I EKG Hx V tach/V fib (arrhythmogenic syncope) Family hx sudden death Inducibility of VT/VF during EP study ICD is the only effective preventive Rx But it also has risk – 8.6%/year
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Case presentation… 40 year old man – hx IVDA heroin
Arrives agitated and c/o back / chest pain An EKG was obtained early --
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Initial EKG
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An old EKG was available
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Case presentation… 40 year old man – hx IVDA heroin
Arrives agitated and c/o back / chest pain An EKG was obtained early – Interventional cardiology was alerted Labs drawn on arrival return --
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…never mind… K+ -- 7.8 mmol/L Cr -- 3.6 mg/dL (1.1 a month earlier)
CPK -- 28,960 Rhabdomyolysis with ATN EKG – Induced Brugada 2° hyperkalemia HyperK+ was aggressively Rx
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Repeat EKG one hour later
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Comparison of EKGs Before Rx After Rx
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Induced Brugada syndrome
Loss of Na+ channel fx with increase temp Reduced inward Na+ current Na+ channel blockers Cocaine, TCA, antihistamines Increased outward K+ current Hyperkalemia
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Induced Brugada-type EKG, a sign for imminent malignant arrhythmias
Junttila M, et al. Circ 117: , 2008 47 pts with induced Brugada EKG Occurred during acute medical care 69% male, mean age 48 years Febrile episode – 16 Drug or medication – 26 Hyperkalemia – 5 24 (51%) developed sx; 18 (38%) with SCD
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Induced Brugada-type EKG, a sign for imminent malignant arrhythmias
Junttila M, et al. Circ 117: , 2008 Drugs and medication Propofol – 7 Flecainide – 5 Propafenone – 3 Atrial fib is prevalent in Brudaga, 11-14%
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Middle-aged man with temp 102.9°
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Fever-induced Brugada pattern: How common is it and what does it mean
Adler A, et al. Heart Rhythm 10: , 2013 Compared EKGs of patients +/- fever 402 pts with fever 909 pts without fever Type I Brugada more common with fever 20X more common 2% vs 0.1% Mean age 47 years / 87% male Persistent Brugada EKG in only 1 patient
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What is the risk? Monitoring and appropriate Rx No one really knows
Brudaga syndrome – low incidence Induced Brugada - ? But the reality exists that someone with induced Brugada will experience SCD Not everyone with fever needs an EKG! But, BrS EKG with known cause needs – Monitoring and appropriate Rx
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