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EKG Rounds

EKG Rounds. Elizabeth Haney 17 August 2006. Case. 26 yo Caucasian male presents to your ED with hx of a single syncopal episode. Witnessed, <1minute, no Seizure activity, alert following event. T 37.3/ HR 80reg/ BP 124/76/ RR 12 O2sat 99%RA/ BG 6.2 Asymptomatic in ED, wants to go home!

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EKG Rounds

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  1. EKG Rounds Elizabeth Haney 17 August 2006

  2. Case • 26 yo Caucasian male presents to your ED with hx of a single syncopal episode. • Witnessed, <1minute, no Seizure activity, alert following event. • T 37.3/ HR 80reg/ BP 124/76/ RR 12 O2sat 99%RA/ BG 6.2 • Asymptomatic in ED, wants to go home! • What else would you like to know?

  3. Additional History • Assoc. with brief episode of palpitations prior to syncope • No SOB, Chest Pain, N/V, HA • PMHx: healthy • Meds: nil • No street drug use • FHx: No known CAD/stroke/seizure/syncope. Father died in his sleep at 34.

  4. His EKG • Any takers on Dx?

  5. Brugada Syndrome Twelve-lead surface EKG showing typical pattern of right bundle-branch block and ST-segment elevation of "coved type" in leads V1 to V3 in a patient identified after a syncopal episode

  6. Brugada Syndrome • First described in 1992 by Pedro and Josep Brugada • New syndrome: assoc. w/ SCD in pts w/ structurally normal hearts & no evidence of CAD • Pts had a distinct set of EKG abnormalities: -RBBB pattern, and persistent ST-segment elevation in the right precordial leads (V1-3).

  7. Epidemiology • Prevalence ranges vary: 0.7-1.0% in Japanese studies, 0.6% in Finnish study, 0.4% in US study. • Male:Female ratio up to 9:1. • More common in Asian populations. Up To Date

  8. Genetics • Autosomal Dominant inheritance • 15% to 30% of patients with the Brugada syndrome, mutations have been found in SCN5A, the cardiac sodium channel α-subunit gene located on chromosome 3

  9. Structural Abnormalities • BS not usually assoc. w/ structural heart dz • Evidence supporting subtle microscopic abnormalities incl. localized myocarditis or microaneurysms. Frustaci et al., Cardiac histological substrate in patients with clinical phenotype of Brugada Sundrome. Circ. 2005 Dec 13;112(24):3680-7. 18 pts in study, 14 w/ RV myocarditis, 7 w/ RV microaneurysms

  10. 3 Patterns of ST Elevation • Type 1: elevated ST segment ( >2 mm) descends with an upward convexity to an inverted T wave. = "coved type" Brugada pattern. • Type 2 and type 3 patterns have a "saddle back" ST-T wave configuration, with the elevated ST segment descends toward the baseline, then rises again to an upright or biphasic T wave. The ST segment is elevated 1 mm in type 2 and <1 mm in type 3.

  11. Brugada Waves: 3 Types ‘Coved’ ST segments w/ T wave inversion • ‘Saddleback’ ST segments • Type 2 • Positive or biphasic T wave • 1mmSTE • Type 3 • Positive T wave • <1mm STE Z Kardiol 2004; 93:784–790 Thanks Dr. Haager

  12. Differential Dx • EKG findings alone not diagnostic. • DDx: • RV pathology • Compression (tumor, hemopericardium) • Inferior MI, RV ischemia, Cardiac contusion • RBBB, LVH • Arrhythmogenic right ventricular cardiomyopathy • Drugs: class IA (procainamide…), IC (propafenone, flecainide…), cocaine, TCA’s, and more • Hyperkalemia, hypercalcemia

  13. Brugada Pattern vs Syndrome • Brugada Pattern = EKG findings without other clinical criteria • Brugada Syndrome = typical EKG findings with other clinical criteria

  14. Clinical Spectrum

  15. Clinical Manifestations • Related to life-threatening ventricular arrhythmias. • SCD occurs in as many as 1/3rd of pts! • Arrhythmic events generally occur between ages of 22-65. • More common at night and during sleep, usually not related to exercise.

  16. Diagnostic Criteria Type I • In evolution. • “Strongly consider in pts that meet the following criteria” • Type I • EKG pattern of type 1 ST segment elevation (coved type) in >1 lead of V1-V3 and 1 of: • Documented VF • Self-terminating polymorphic VT • Family Hx of SCD <45 • Other Family members w/ Brugada ECG pattern • EPS inducibility of VT • Unexplained syncope suggestive of a tachyarrhythmia • Nocturnal agonal respirations Wilde et al. Eur Heart J 2002;23:1648

  17. Diagnostic Criteria Type 2 + 3 • Type 2 or 3 • EKG pattern of type 2 or 3 ST segment elevation (saddle-back type) in >1 lead of V1-V3, with conversion to type 1 following challenge w/ a sodium channel blocker And1 of: • Documented VF • Self-terminating polymorphic VT • Family Hx of SCD <45 • Other Family members w/ Brugada ECG pattern • EPS inducibility of VT • Unexplained syncope suggestive of a tachyarrhythmia • Nocturnal agonal respirations

  18. Proposed Work-Up

  19. Treatment • Refer for EP studies • ICD placement • When provided with an ICD, mortality at 10-year follow-up has been 0%. • Quinidine (sodium channel blocker) research shows promise. Currently may have role for pts with ICD and frequent discharges (consider amiodarone, quinidine or hydroquinidine). Uptodate.com

  20. Take Home • Be familiar with the EKG manifestations of Brugada syndrome to ensure early diagnosis and prompt referral.

  21. References • Zipes: Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 7th ed. • Brugada P, Brugada J: Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. J Am Coll Cardiol 1992;20:1391-1396 • Littmann et al., Brugada syndrome and “Brugada sign”: Clinical spectrum with a guide for the clinician ,American Heart Journal, 145;768-778 • Frustaci et al., Cardiac histological substrate in patients with clinical phenotype of Brugada Sundrome. Circ. 2005 Dec 13;112(24):3680-7. • Wilde et al., Proposed diagnostic criteria for the Brugada syndrome. Eur Heart J 2002; 23:1648 • Mattu et al., The Brugada Syndrome, Am J Emerg Med 2003;21:146-151 • Uptodate.com • J.,R., and P. Brugada, Determinants of Sudden Cardiac Death in Individuals With the Electrocardiographic Pattern of Brugada Syndrome and No Previous Cardiac Arrest. Circ. Dec. 2003; 108: 3092-3096

  22. Pts w/ ECG diagnostic of Brugada syndrome and no previous cardiac arrest have an 8% risk of SCD during a short-term follow-up period of 2 years. • Lowest-risk group = no syncopal episodes, diagnostic ECG only with drug challenge, and noninducibility during programmed ventricular stimulation (0.5% incidence of events). • Highest-risk group = combo of a previous history of syncope, a spontaneously abnormal ECG, and inducible sustained arrhythmias during programmed ventricular stimulation (27.2% incidence of events). J.,R., and P. Brugada, Determinants of Sudden Cardiac Death in Individuals With the Electrocardiographic Pattern of Brugada Syndrome and No Previous Cardiac Arrest. Circ. Dec. 2003; 108: 3092-3096

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