sudden cardiac arrest brugada syndrome

89
Sudden Cardiac Arrest BRUGADA SYNDROME Carlo Francisco S. Gochuico, M.D. August 14, 2008

Upload: yank

Post on 24-Feb-2016

121 views

Category:

Documents


3 download

DESCRIPTION

Sudden Cardiac Arrest BRUGADA SYNDROME. Carlo Francisco S. Gochuico, M.D. August 14, 2008. OBJECTIVES. To present a case of sudden cardiac arrest in a young male To discuss the approach and management of Brugada syndrome. General Data. J.D. 28 year old Male Filipino Chief Complaint - PowerPoint PPT Presentation

TRANSCRIPT

Page 1: Sudden Cardiac Arrest  BRUGADA SYNDROME

Sudden Cardiac Arrest BRUGADA SYNDROME

Carlo Francisco S. Gochuico, M.D.

August 14, 2008

Page 2: Sudden Cardiac Arrest  BRUGADA SYNDROME

OBJECTIVES

• To present a case of sudden cardiac arrest in a young male

• To discuss the approach and management of Brugada syndrome

Page 3: Sudden Cardiac Arrest  BRUGADA SYNDROME

General Data

• J.D.• 28 year old• Male• Filipino

Chief ComplaintLoss of consciousness

Page 4: Sudden Cardiac Arrest  BRUGADA SYNDROME

History of Present History of Present IllnessIllness

One hour PTAOne hour PTA Found unconsciousFound unconscious Seen with upward rolling of Seen with upward rolling of

eyeballs, stiffening of eyeballs, stiffening of extremities, and salivary poolingextremities, and salivary pooling

Lasted 3 to 5 minutes Lasted 3 to 5 minutes Regained full consciousness with Regained full consciousness with

no recollection of the incidentno recollection of the incident Was able to drink, sit on a chair, Was able to drink, sit on a chair,

and talk with parentsand talk with parents No slurring of speech, no No slurring of speech, no

extremity weakness, no chest extremity weakness, no chest painpain

Page 5: Sudden Cardiac Arrest  BRUGADA SYNDROME

History of Present History of Present IllnessIllness

Five minutes Five minutes after after

Recurrence of Recurrence of stiffening of stiffening of extremities and extremities and upward rolling of upward rolling of eyeballseyeballs

Rushed to MMC ERRushed to MMC ER During transit, he During transit, he

remained remained unconsciousunconscious

Page 6: Sudden Cardiac Arrest  BRUGADA SYNDROME

Events at EREvents at ER Quicklook showed Quicklook showed

cyanosiscyanosis BP 0 HR 0BP 0 HR 0 GCS 3 E1V1M1 GCS 3 E1V1M1 Initial tracing at ER at Initial tracing at ER at

1:42 AM1:42 AM Chest compressions Chest compressions

and bag mask and bag mask ventilationventilation

Defibrillation at 200 Defibrillation at 200 joulesjoules

Epinephrine 1mg IVEpinephrine 1mg IV Intubated and advised Intubated and advised

ICU admission ICU admission CXRAYCXRAY, , stat5stat5 and ABG and ABG

Page 7: Sudden Cardiac Arrest  BRUGADA SYNDROME

12 L EKG 8-1-08 2:16 AM post defibrillation 12 L EKG 8-1-08 2:16 AM post defibrillation CRBBB CRBBB PR interval 0.16 sec PR interval 0.16 sec QT interval 0.36 secQT interval 0.36 sec

Page 8: Sudden Cardiac Arrest  BRUGADA SYNDROME

Events at EREvents at ER 2:33 AM at 2:33 AM at

ERER Systolic Systolic

BP170BP170 Pulse 80s Pulse 80s

regularregular Post CP arrestPost CP arrest

Page 9: Sudden Cardiac Arrest  BRUGADA SYNDROME

Events at EREvents at ER 2:47 AM 2:47 AM Episode of Episode of

stiffening of stiffening of extremitiesextremities

chest chest compression compression and and defibrillation defibrillation at 200 at 200 joulesjoules

Page 10: Sudden Cardiac Arrest  BRUGADA SYNDROME

Events at EREvents at ER 2:52 AM 2:52 AM Post defibrillationPost defibrillation BP 120/80BP 120/80 Pulse 80sPulse 80s Amiodarone drip Amiodarone drip

startedstarted Cardiac enzymesCardiac enzymes Referred to Referred to

neurologyneurology Admitted to ICUAdmitted to ICU

Page 11: Sudden Cardiac Arrest  BRUGADA SYNDROME

Review of SystemsReview of Systems

General: General: no feverno fever, fatigue, weight loss, fatigue, weight loss Skin: no rashes, jaundice, ecchymoses, Skin: no rashes, jaundice, ecchymoses,

petechiaepetechiae Head: no recent head injury, headache, Head: no recent head injury, headache,

dizzinessdizziness Eyes: no blurring of vision, redness, painEyes: no blurring of vision, redness, pain Ears: no tinnitus, vertigo, earache, Ears: no tinnitus, vertigo, earache,

dischargedischarge

Page 12: Sudden Cardiac Arrest  BRUGADA SYNDROME

Review of SystemsReview of Systems Nose: no colds, nasal congestion, Nose: no colds, nasal congestion,

dischargedischarge Mouth: no sore throat, hoarsenessMouth: no sore throat, hoarseness Neck: no pain, lumps, mass, stiffnessNeck: no pain, lumps, mass, stiffness Respiratory: no cough, dyspnea, Respiratory: no cough, dyspnea,

wheezing, hemoptysiswheezing, hemoptysis Cardiac: no chest pain, palpitations, Cardiac: no chest pain, palpitations,

orthopnea, PND, edema, recent chest orthopnea, PND, edema, recent chest traumatrauma

Page 13: Sudden Cardiac Arrest  BRUGADA SYNDROME

Review of SystemsReview of Systems Gastrointestinal: no nausea, vomiting, Gastrointestinal: no nausea, vomiting,

regurgitation, dysphagia, regurgitation, dysphagia, hematemesis, abdominal pain, change hematemesis, abdominal pain, change in frequency and characteristic of in frequency and characteristic of stoolsstools

Urinary: no hematuria, dysuria, Urinary: no hematuria, dysuria, oliguria, polyuria, urgency, hesitancyoliguria, polyuria, urgency, hesitancy

Vascular: no claudication, varicose Vascular: no claudication, varicose veins, leg crampsveins, leg cramps

Musculoskeletal: no myalgia, Musculoskeletal: no myalgia, arthralgia, and swellingarthralgia, and swelling

Page 14: Sudden Cardiac Arrest  BRUGADA SYNDROME

Review of SystemsReview of Systems Hematologic: no anemia, pallor, Hematologic: no anemia, pallor,

easy bruising or bleedingeasy bruising or bleeding Endocrine: no heat or cold Endocrine: no heat or cold

intolerance, no excessive thirst or intolerance, no excessive thirst or hungerhunger

Psychiatric: no depression, Psychiatric: no depression, nervousnessnervousness

Page 15: Sudden Cardiac Arrest  BRUGADA SYNDROME

Past Medical HistoryPast Medical History No history of HPN, DM, and BANo history of HPN, DM, and BA PTB, treated for 6 monthsPTB, treated for 6 months No previous seizure disorder, no No previous seizure disorder, no

known neurologic and cardiac known neurologic and cardiac problemsproblems

Had a history of syncope during Had a history of syncope during early childhood and another one a early childhood and another one a year ago, no work-ups were doneyear ago, no work-ups were done

Page 16: Sudden Cardiac Arrest  BRUGADA SYNDROME

Family HistoryFamily History (+) HPN in paternal side(+) HPN in paternal side (-) DM, BA, stroke, cancer(-) DM, BA, stroke, cancer No seizure disorderNo seizure disorder

Page 17: Sudden Cardiac Arrest  BRUGADA SYNDROME

Personal and Social Personal and Social HistoryHistory Occasional smokerOccasional smoker Occasional alcoholic beverage Occasional alcoholic beverage

drinkerdrinker Denies illicit drug useDenies illicit drug use

Page 18: Sudden Cardiac Arrest  BRUGADA SYNDROME

PHYSICAL PHYSICAL EXAMINATIONEXAMINATION

Conscious, restless, intubatedConscious, restless, intubated BP 120/80 HR 80 regular RR 20 BP 120/80 HR 80 regular RR 20

assisted afebrileassisted afebrile Warm moist skin, no active Warm moist skin, no active

dermatoses, no jaundicedermatoses, no jaundice Anicteric sclerae, pink palpebral Anicteric sclerae, pink palpebral

conjunctivae, pupils 2-3 mm ERTL OUconjunctivae, pupils 2-3 mm ERTL OU No visible anterior neck mass, no neck No visible anterior neck mass, no neck

vein distention, no carotid bruitvein distention, no carotid bruit No cervical lymphadenopathiesNo cervical lymphadenopathies

Page 19: Sudden Cardiac Arrest  BRUGADA SYNDROME

PHYSICAL PHYSICAL EXAMINATIONEXAMINATION

Equal chest expansion, no retractions, no Equal chest expansion, no retractions, no rales, wheezes, cracklesrales, wheezes, crackles

Adynamic precordium, AB at 5th LICS MCL, Adynamic precordium, AB at 5th LICS MCL, regular rhythm, S1>S2 at base, S2>S1 at regular rhythm, S1>S2 at base, S2>S1 at apex, no extra heart sounds, no murmursapex, no extra heart sounds, no murmurs

Abdomen flabby, normoactive bowel sounds, Abdomen flabby, normoactive bowel sounds, soft, no guarding, no direct and rebound soft, no guarding, no direct and rebound tenderness, no hepatosplenomegalytenderness, no hepatosplenomegaly

No costovertebral angle tendernessNo costovertebral angle tenderness No pedal edemaNo pedal edema Pulses full and equalPulses full and equal

Page 20: Sudden Cardiac Arrest  BRUGADA SYNDROME

PHYSICAL PHYSICAL EXAMINATIONEXAMINATION

GCS 11 E4V1 (intubated) M6GCS 11 E4V1 (intubated) M6 No papilledemaNo papilledema Full and equal EOMFull and equal EOM No facial asymmetryNo facial asymmetry Intact doll’s eye movement, gag and corneal Intact doll’s eye movement, gag and corneal

reflexesreflexes Direct tendon reflexes normalDirect tendon reflexes normal Motor and Sensory: intactMotor and Sensory: intact Localizes to pain and temperatureLocalizes to pain and temperature No babinski, kernig’s, and brudzinskiNo babinski, kernig’s, and brudzinski

Page 21: Sudden Cardiac Arrest  BRUGADA SYNDROME

Salient FeaturesSalient Features 28 M Filipino28 M Filipino Loss of consciouness Loss of consciouness

at night during sleepat night during sleep 2 episodes of 2 episodes of

stiffening of stiffening of extremitiesextremities

Post CP arrest 2x Post CP arrest 2x (VFib)(VFib)

No fever, headache, No fever, headache, chest pain, chest pain, weaknessweakness

History of syncope History of syncope 2x2x

Positive family Positive family history of cardiac history of cardiac diseasedisease

No illicit drug useNo illicit drug use

Page 22: Sudden Cardiac Arrest  BRUGADA SYNDROME

Differential DiagnosisDifferential Diagnosis

Loss of consciousness

Neurologic Cardiac

Structural Primary physiologic abnormalitySeizure Disorder Cerebrovascular Accident

Page 23: Sudden Cardiac Arrest  BRUGADA SYNDROME

Differential DiagnosisDifferential Diagnosis

Neurologic

Seizure Cerebrovascular accident

No previous historyCBC electrolytes

glucose toxicology liver and

renal function test CT, EEG

Imaging (CT)

Page 24: Sudden Cardiac Arrest  BRUGADA SYNDROME

Loss of consciousness

Neurologic Cardiac

Structural Primary physiologic abnormalitySeizure Disorder Cerebrovascular Accident

Page 25: Sudden Cardiac Arrest  BRUGADA SYNDROME

Differential DiagnosisDifferential Diagnosis

Cardiac

Structural heart disease Non-structural heart disease

Page 26: Sudden Cardiac Arrest  BRUGADA SYNDROME

Differential DiagnosisDifferential Diagnosis Structural heart diseasesStructural heart diseases

– Ischemic heart diseaseIschemic heart disease– Non-ischemic cardiomyopathiesNon-ischemic cardiomyopathies– Valvular heart diseasesValvular heart diseases– Arrhythmogenic right ventricular Arrhythmogenic right ventricular

dysplasiadysplasia Primary electrophysiologic abnormalitiesPrimary electrophysiologic abnormalities

– Long QT syndromeLong QT syndrome– Brugada syndromeBrugada syndrome

Page 27: Sudden Cardiac Arrest  BRUGADA SYNDROME

IschemiaIschemia

Cardiac arrest due to ventricular Cardiac arrest due to ventricular arrhythmias may be due to arrhythmias may be due to chronic scar or to acute chronic scar or to acute MI/ischemia. A chronic infarct scar MI/ischemia. A chronic infarct scar can serve as the focus for can serve as the focus for reentrant ventricular reentrant ventricular tachyarrhythmiastachyarrhythmias

Page 28: Sudden Cardiac Arrest  BRUGADA SYNDROME

Non-ischemic Non-ischemic cardiomyopathiescardiomyopathies

represent the second largest group represent the second largest group of patients who experience SCD of patients who experience SCD

Dilated cardiomyopathy is usually Dilated cardiomyopathy is usually characterized by ventricular characterized by ventricular dilatation, initially usually of the dilatation, initially usually of the left ventricle (LV), with myocyte left ventricle (LV), with myocyte hypertrophy and diminished hypertrophy and diminished systolic function systolic function

Page 29: Sudden Cardiac Arrest  BRUGADA SYNDROME

Hypertrophic Hypertrophic cardiomyopathy (HCM)cardiomyopathy (HCM)

an autosomal-dominant, an autosomal-dominant, incompletely penetrant genetic incompletely penetrant genetic disorder resulting from a mutation disorder resulting from a mutation in one of the many (>45) genes in one of the many (>45) genes encoding proteins of the cardiac encoding proteins of the cardiac muscle sarcomere muscle sarcomere

Page 30: Sudden Cardiac Arrest  BRUGADA SYNDROME

Echo of HCMEcho of HCM Small LV cavity due to marked Small LV cavity due to marked

hypertrophy of the myocardium and hypertrophy of the myocardium and encroachment into the LV cavity encroachment into the LV cavity

Reduced septal motion and thickening Reduced septal motion and thickening during systole, particularly of the upper during systole, particularly of the upper septum, due to the disarray of the septum, due to the disarray of the myofibrillar architecture and abnormal myofibrillar architecture and abnormal contractile function contractile function

Reduced rate of closure of the mitral Reduced rate of closure of the mitral valve in mid diastole due to a decrease in valve in mid diastole due to a decrease in LV compliance Left atrial enlargementLV compliance Left atrial enlargement

Page 31: Sudden Cardiac Arrest  BRUGADA SYNDROME

Cardiac

Structural heart diseaseNon-structural heart disease

Primary physiologic abnormality

Page 32: Sudden Cardiac Arrest  BRUGADA SYNDROME

Course in the WardCourse in the Ward 11stst Hospital day in the ICU (1230 PM) Hospital day in the ICU (1230 PM) Repeat 12L EKG doneRepeat 12L EKG done

– Rsr’ patternRsr’ pattern– ST elevation in lead V1-V3ST elevation in lead V1-V3

2D echo – N LVD EF69% Normal LA and RA 2D echo – N LVD EF69% Normal LA and RA dimensionsdimensions

Normal MV, TV, AV, PVNormal MV, TV, AV, PV Mild MR, TRMild MR, TR Normal left ventricular diastolic function Normal left ventricular diastolic function

indicesindices

FIo2 adjusted to 0.35FIo2 adjusted to 0.35

Page 33: Sudden Cardiac Arrest  BRUGADA SYNDROME

Course in the WardCourse in the Ward ICU at 1500HICU at 1500H T-piece placed and repeat ABG T-piece placed and repeat ABG

donedone Referred to cardiology EPSReferred to cardiology EPS Thyroid function test requestedThyroid function test requested Quinidine bisulfate startedQuinidine bisulfate started

Page 34: Sudden Cardiac Arrest  BRUGADA SYNDROME

Course in the WardCourse in the Ward ICU at 1750 HICU at 1750 H Patient extubatedPatient extubated

44thth Hospital day Hospital day Transferred to regular roomTransferred to regular room Quinidine 200mg/tab adjusted to Quinidine 200mg/tab adjusted to

1 ½ tablets in the morning and 1 ½ tablets in the morning and evening, and 1 tablet at lunchtime evening, and 1 tablet at lunchtime

Page 35: Sudden Cardiac Arrest  BRUGADA SYNDROME

Course in the WardCourse in the Ward 55thth hospital day hospital day Repeat 12 L EKGRepeat 12 L EKG

– NormalNormal

Discharged on 6Discharged on 6thth hospital day hospital day Follow-up after 1 weekFollow-up after 1 week

Page 36: Sudden Cardiac Arrest  BRUGADA SYNDROME

• CXRAY Aug 1, 2008

• Lungs are clear

• Heart is magnified

• ET in place with tip at carina

Page 37: Sudden Cardiac Arrest  BRUGADA SYNDROME

• Cranial CT Scan Aug 1, 08

• Normal non-contrast CT of the brain

• EEG• Normal

Page 38: Sudden Cardiac Arrest  BRUGADA SYNDROME

2D 2D ECHOCARDIOGRAPHYECHOCARDIOGRAPHYAugust 1, 2008August 1, 2008 Normal left ventricular dimension with Normal left ventricular dimension with

normal wall motion and contractility.normal wall motion and contractility. Computed LV EF 69%Computed LV EF 69% Normal left and right atrial Normal left and right atrial

dimensionsdimensions Normal mitral, aortic, tricuspid, and Normal mitral, aortic, tricuspid, and

pulmonic valvespulmonic valves Mild MR, TRMild MR, TR Normal left ventricular diastolic Normal left ventricular diastolic

function indicesfunction indices

Page 39: Sudden Cardiac Arrest  BRUGADA SYNDROME

Repeat 12 L EKG 8-1-08 12:30 PM Repeat 12 L EKG 8-1-08 12:30 PM IRBBBIRBBBPR interval 0.16 secPR interval 0.16 secQT interval 0.40 secQT interval 0.40 sec

Page 40: Sudden Cardiac Arrest  BRUGADA SYNDROME

Laboratory Results and Ancillary Laboratory Results and Ancillary ProceduresProcedures

CBCCBC Hgb 16.8Hgb 16.8 Hct 50.2Hct 50.2 WBC 14.21WBC 14.21

– seg54 seg54 – lymph36 lymph36 – eos4 eos4 – mono5 mono5 – Baso1Baso1

Platelet 204000Platelet 204000

Stat 5Stat 5 Na 137Na 137 K 2.7K 2.7 Hgb 16.7Hgb 16.7 Hct 49Hct 49 Glucose Glucose

(random) 245 (random) 245 mg/dLmg/dL

Page 41: Sudden Cardiac Arrest  BRUGADA SYNDROME

Laboratory Results and Ancillary Laboratory Results and Ancillary ProceduresProcedures

PTTPTT patient patient 27.327.3 controlcontrol 27.6 27.6

PTPT patient patient 11.111.1 control control 11.711.7 activity activity 115.6%115.6% INR INR 0.940.94

Cardiac enzymesCardiac enzymesCK total 165 U/LCK total 165 U/LCPK MB 1.4 ng/mLCPK MB 1.4 ng/mLTroponin I 0 ng/mLTroponin I 0 ng/mL

Page 42: Sudden Cardiac Arrest  BRUGADA SYNDROME

Laboratory Results and Ancillary ProceduresLaboratory Results and Ancillary Procedures

glucose 120 glucose 120 CPK 506 CPK 506 LDH 262LDH 262 ALT 185 ALT 185 AST 82 AST 82 alkaline alkaline

phosphatase 148phosphatase 148

SPEC 23SPEC 23 Na 137 Na 137 K 3.6 K 3.6 Cl 103 Cl 103 calcium 9.78 calcium 9.78 BUN 13 BUN 13 creatinine 1.1creatinine 1.1 HDL 46 HDL 46 trig 119 trig 119 LDL 166 LDL 166 cholesterol 245cholesterol 245

Page 43: Sudden Cardiac Arrest  BRUGADA SYNDROME

Laboratory Results and Ancillary Laboratory Results and Ancillary ProceduresProcedures Routine urinalysisRoutine urinalysis Yellow hazy Yellow hazy PH 6 PH 6 spgr 1.020 spgr 1.020 +3 protein +3 protein trace sugar trace sugar negative ketones, negative ketones,

nitrites, leucocyte nitrites, leucocyte esterase esterase

+1 blood +1 blood rbc 2/hpf rbc 2/hpf wbc 2/hpf wbc 2/hpf epith 10/hpfepith 10/hpf Bact 15/hpfBact 15/hpf

Thyroid Function Thyroid Function TestTest

TSHTSH 0.0370.037 (0.27-(0.27-3.75)3.75)

FT3FT3 4.419 (4.2-12)4.419 (4.2-12) FT4FT4 17.815 (8.8-33)17.815 (8.8-33)

Page 44: Sudden Cardiac Arrest  BRUGADA SYNDROME

Laboratory Results and Ancillary Laboratory Results and Ancillary ProceduresProcedures

ABGABG Post intubation Post intubation PO2PO2 426.2 426.2 PHPH 7.34 7.34 PCO2PCO2 41 41 HCO3HCO3 22.1 22.1 O2 satO2 sat 99.8 99.8 AC mode 100% AC mode 100%

FiO2 VT 420FiO2 VT 420

Page 45: Sudden Cardiac Arrest  BRUGADA SYNDROME

Laboratory Results and Ancillary Laboratory Results and Ancillary ProceduresProcedures

ABGABG

5 PM 5 PM PO2PO2 169.1 169.1 PHPH 7.39 7.39 PCO2PCO2 37.3 37.3 HCO3HCO3 22.5 22.5 O2 satO2 sat 99.1 99.1 inline neb via T piece 35% FiO2inline neb via T piece 35% FiO2

Page 46: Sudden Cardiac Arrest  BRUGADA SYNDROME

Repeat 12 L EKG 8-6-08 9:54 AM Repeat 12 L EKG 8-6-08 9:54 AM NormalNormalPR interval 0.20 secPR interval 0.20 secQT interval 0.44 secQT interval 0.44 sec

Page 47: Sudden Cardiac Arrest  BRUGADA SYNDROME

DISCUSSIONDISCUSSION Sudden cardiac death (SCD) is an Sudden cardiac death (SCD) is an

unexpected death due to cardiac unexpected death due to cardiac causes, heralded by loss of causes, heralded by loss of consciousness occurring in a short consciousness occurring in a short time period (generally within 1 hour of time period (generally within 1 hour of symptom onset)symptom onset)

Most cases are due to cardiac Most cases are due to cardiac arrhythmias such as VF or VT which is arrhythmias such as VF or VT which is responsible for 50-80% of casesresponsible for 50-80% of cases

Page 48: Sudden Cardiac Arrest  BRUGADA SYNDROME

Sudden Cardiac DeathSudden Cardiac Death Most cases of SCD occur in patients with Most cases of SCD occur in patients with

structural abnormalities in the heart, structural abnormalities in the heart, related to either a prior MI, coronary related to either a prior MI, coronary artery disease, cardiomyopathiesartery disease, cardiomyopathies

Valvular diseases such as aortic stenosis Valvular diseases such as aortic stenosis are associated with increased risk of SCDare associated with increased risk of SCD

Acute inflammatory and infiltrative Acute inflammatory and infiltrative disorders, such as myocarditis, provide a disorders, such as myocarditis, provide a sustained risk of SCDsustained risk of SCD

Page 49: Sudden Cardiac Arrest  BRUGADA SYNDROME

Sudden Cardiac DeathSudden Cardiac Death Less commonly, SCD happens in Less commonly, SCD happens in

patients who may not have patients who may not have apparent structural diseaseapparent structural disease

These conditions are usually These conditions are usually inherited arrhythmia syndromes inherited arrhythmia syndromes or primary electrophysiologic or primary electrophysiologic abnormalitiesabnormalities

Page 50: Sudden Cardiac Arrest  BRUGADA SYNDROME

Primary Primary Electrophysiologic Electrophysiologic AbnormalitiesAbnormalities This generally represents a group This generally represents a group

of abnormalities in which patients of abnormalities in which patients have no apparent structural heart have no apparent structural heart disease but have a primary disease but have a primary electrophysiologic abnormality electrophysiologic abnormality that predisposes them to VF or VTthat predisposes them to VF or VT

Brugada syndrome, Long QT Brugada syndrome, Long QT syndromesyndrome

Page 51: Sudden Cardiac Arrest  BRUGADA SYNDROME

Brugada SyndromeBrugada Syndrome A cardiac disease caused by an A cardiac disease caused by an

inherited ion channelopathy inherited ion channelopathy associated with a propensity to associated with a propensity to develop ventricular fibrillation (VF)develop ventricular fibrillation (VF)

Reported as early as 1953 but was Reported as early as 1953 but was first described as a distinct clinical first described as a distinct clinical entity associated with a high risk entity associated with a high risk of sudden cardiac death in 1992of sudden cardiac death in 1992

Page 52: Sudden Cardiac Arrest  BRUGADA SYNDROME

Brugada SyndromeBrugada Syndrome In the 1980s, the Centers for Disease In the 1980s, the Centers for Disease

Control and Prevention reported a high Control and Prevention reported a high incidence of sudden death in young incidence of sudden death in young immigrants from Southeast Asia.immigrants from Southeast Asia.

For natives, it is known as For natives, it is known as lai tai lai tai (death (death during sleep) in Thailand, during sleep) in Thailand, bangungut bangungut ((sudden death during sleep) in the sudden death during sleep) in the Philippines, and Philippines, and pokkuri pokkuri (unexpected (unexpected sudden death at night) in Japansudden death at night) in Japan

Page 53: Sudden Cardiac Arrest  BRUGADA SYNDROME

Brugada SyndromeBrugada Syndrome Familial disease with an autosomal Familial disease with an autosomal

dominant mode of transmission, with dominant mode of transmission, with incomplete penetrance and an incidence incomplete penetrance and an incidence ranging between 5 and 66 per 10 000ranging between 5 and 66 per 10 000

In Southeast Asia where it is endemic, it is In Southeast Asia where it is endemic, it is distinguished by a male predominance distinguished by a male predominance (8:1 male:female ratio) and the (8:1 male:female ratio) and the appearance of arrhythmic events at an appearance of arrhythmic events at an average age of 40 years (range: 1 to 77 average age of 40 years (range: 1 to 77 years)years)

Page 54: Sudden Cardiac Arrest  BRUGADA SYNDROME

Brugada SyndromeBrugada Syndrome The syndrome typically manifests The syndrome typically manifests

during adulthood, with a mean age of during adulthood, with a mean age of sudden death of 41 +/- 15 years. The sudden death of 41 +/- 15 years. The youngest patient clinically diagnosed youngest patient clinically diagnosed with the syndrome is 2 days old and with the syndrome is 2 days old and the oldest is 84 years oldthe oldest is 84 years old

Brugada Syndrome: Report of the Second Consensus Conference Circulation 2005

Page 55: Sudden Cardiac Arrest  BRUGADA SYNDROME

Brugada SyndromeBrugada Syndrome It is thought that it may cause 4 to 10 It is thought that it may cause 4 to 10

sudden deaths per 10,000 inhabitants in sudden deaths per 10,000 inhabitants in Southeast Asia annually, making it the Southeast Asia annually, making it the second most common cause of natural second most common cause of natural death in men aged younger than 40 death in men aged younger than 40 yearsyears

In endemic countries, it has been In endemic countries, it has been estimated to cause at least 4% of all estimated to cause at least 4% of all sudden deaths and at least 20% of all sudden deaths and at least 20% of all sudden cardiac deaths in patients with sudden cardiac deaths in patients with structurally normal hearts.structurally normal hearts.

Orphanet Journal of Rare Diseases 2006

Page 56: Sudden Cardiac Arrest  BRUGADA SYNDROME

Brugada SyndromeBrugada Syndrome The prevalence of BrS in the general The prevalence of BrS in the general

population is unknown. The population is unknown. The suggested prevalence ranges from suggested prevalence ranges from 5/1,000 (Caucasians) to 14/1,000 5/1,000 (Caucasians) to 14/1,000 (Japanese)(Japanese)

In Laos, it causes an estimated 1 In Laos, it causes an estimated 1 sudden death per 1000 inhabitants sudden death per 1000 inhabitants per year, and in Thailand, per year, and in Thailand, unexpected sudden death is the unexpected sudden death is the most common cause of natural death most common cause of natural death in young peoplein young people

Page 57: Sudden Cardiac Arrest  BRUGADA SYNDROME

Brugada SyndromeBrugada Syndrome It has been linked to a genetic mutation It has been linked to a genetic mutation

located on the located on the SCN5A SCN5A gene on chromosome 3 gene on chromosome 3 p21-23, which codes for the α subunit of the p21-23, which codes for the α subunit of the cardiomyocyte sodium ion channelscardiomyocyte sodium ion channels

This mutation leads to either complete loss of This mutation leads to either complete loss of channel function or an accelerated recovery channel function or an accelerated recovery from activationfrom activation

This can generate heterogeneity of This can generate heterogeneity of repolarization and increase the chance of repolarization and increase the chance of intramyocardial re-entry circuits, which may intramyocardial re-entry circuits, which may induce ventricular tachyarrhythmiasinduce ventricular tachyarrhythmias

Page 58: Sudden Cardiac Arrest  BRUGADA SYNDROME

Genetic mutations detected so far in Genetic mutations detected so far in the Brugada syndrome result in the Brugada syndrome result in defective myocardial sodium channels defective myocardial sodium channels that reduce sodium inflow currents, that reduce sodium inflow currents, resulting in shorter-than-normal action resulting in shorter-than-normal action potentialspotentials

Page 59: Sudden Cardiac Arrest  BRUGADA SYNDROME

Brugada SyndromeBrugada Syndrome It can also present atrial It can also present atrial

fibrillation, which is present in 10% fibrillation, which is present in 10% to 20% of casesto 20% of cases

Potential clinical manifestations Potential clinical manifestations dizziness, palpitations, syncope, dizziness, palpitations, syncope, and sudden cardiac deathand sudden cardiac death

ECG abnormalities constitute the ECG abnormalities constitute the hallmark of Brugada syndromehallmark of Brugada syndrome

Page 60: Sudden Cardiac Arrest  BRUGADA SYNDROME

Circadian patternCircadian patternVF and sudden death in Brugada syndrome usually occur at rest and at night

Page 61: Sudden Cardiac Arrest  BRUGADA SYNDROME

Modulating and precipitating Modulating and precipitating factorsfactors

The ECG manifestations of congenital The ECG manifestations of congenital Brugada syndrome are often concealed Brugada syndrome are often concealed but can be unmasked or modulated by:but can be unmasked or modulated by:

Sodium channel blockers, a febrile Sodium channel blockers, a febrile state, vagotonic agents, alpha and state, vagotonic agents, alpha and beta adrenergic agonists, tricyclic or beta adrenergic agonists, tricyclic or tetracyclic antidepressants, a tetracyclic antidepressants, a combination of glucose and insulin, combination of glucose and insulin, hyperkalemia, hypokalemia, hyperkalemia, hypokalemia, hypercalcemia, and alcohol and hypercalcemia, and alcohol and cocaine toxicitycocaine toxicity

Page 62: Sudden Cardiac Arrest  BRUGADA SYNDROME

Brugada SyndromeBrugada Syndrome Electrocardiographic CharacteristicsElectrocardiographic Characteristics

The J wave is a deflection that appears in the ECG as a late delta wave following the QRS or as a small secondary R wave (R'). Also referred to as the Osborn wave

Page 63: Sudden Cardiac Arrest  BRUGADA SYNDROME

Brugada SyndromeBrugada Syndrome Electrocardiographic CharacteristicsElectrocardiographic Characteristics

Type1 is characterized Type1 is characterized by a prominent coved by a prominent coved ST-segment elevation ST-segment elevation displaying J wave displaying J wave amplitude or ST-amplitude or ST-segment elevation 2 segment elevation 2 mm or 0.2 mV at its mm or 0.2 mV at its peak followed by a peak followed by a negative T-wave, with negative T-wave, with little or no isoelectric little or no isoelectric separationseparation

Proposed Diagnostic Criteria for the Brugada Syndrome: Consensus Report Circulation 2002

Page 64: Sudden Cardiac Arrest  BRUGADA SYNDROME

Brugada SyndromeBrugada Syndrome

Page 65: Sudden Cardiac Arrest  BRUGADA SYNDROME

Brugada SyndromeBrugada Syndrome Electrocardiographic CharacteristicsElectrocardiographic Characteristics

Type 2 also has a high Type 2 also has a high take-off STsegment take-off STsegment elevation, but in this elevation, but in this case, J wave amplitude case, J wave amplitude (2 mm) gives rise to a (2 mm) gives rise to a gradually descending gradually descending ST-segment elevation ST-segment elevation (remaining 1 mm above (remaining 1 mm above the baseline), followed the baseline), followed by a positive or biphasic by a positive or biphasic T-wave that results in a T-wave that results in a saddle back saddle back configurationconfiguration

Proposed Diagnostic Criteria for the Brugada Syndrome: Consensus ReportCirculation 2002

Page 66: Sudden Cardiac Arrest  BRUGADA SYNDROME

Brugada SyndromeBrugada Syndrome Electrocardiographic CharacteristicsElectrocardiographic Characteristics Type 3 is a right Type 3 is a right

precordial ST-precordial ST-segment segment elevation of 1 elevation of 1 mm of saddle mm of saddle back type, back type, coved type, or coved type, or bothboth

Proposed Diagnostic Criteria for the Brugada Syndrome: Consensus ReportCirculation 2002

Page 67: Sudden Cardiac Arrest  BRUGADA SYNDROME

Brugada SyndromeBrugada Syndrome

Proposed Diagnostic Criteria for the Brugada Syndrome: Consensus ReportCirculation 2002

Page 68: Sudden Cardiac Arrest  BRUGADA SYNDROME

Brugada Syndrome: Report of the Brugada Syndrome: Report of the Second Consensus Conference Second Consensus Conference Circulation Circulation 20022002 It can be established in the presence of:It can be established in the presence of: ST-segment elevation (type 1) in more than one ST-segment elevation (type 1) in more than one

right precordial lead (V1 to V3), and one of the right precordial lead (V1 to V3), and one of the following:following:

Documented ventricular fibrillation; self Documented ventricular fibrillation; self terminating polymorphic ventricular tachycardia; terminating polymorphic ventricular tachycardia; a family history of sudden cardiac death (<45 a family history of sudden cardiac death (<45 years); coved type ECGs in family members; years); coved type ECGs in family members; electrophysiological inducibility; syncopeelectrophysiological inducibility; syncope

There should be no other factors that can There should be no other factors that can account for the ECG abnormalityaccount for the ECG abnormality

Page 69: Sudden Cardiac Arrest  BRUGADA SYNDROME

Brugada Syndrome: Report of the Brugada Syndrome: Report of the Second Consensus ConferenceSecond Consensus Conference

Appearance of type 2 ST-segment Appearance of type 2 ST-segment elevation ("saddleback type") in more elevation ("saddleback type") in more than one right precordial lead upon than one right precordial lead upon challenge with a sodium channel challenge with a sodium channel blocker. blocker.

A drug-induced ST-segment elevation to A drug-induced ST-segment elevation to a value >2 mm should raise the a value >2 mm should raise the possibility of Brugada syndrome when possibility of Brugada syndrome when one or more clinical criteria are presentone or more clinical criteria are present

Page 70: Sudden Cardiac Arrest  BRUGADA SYNDROME

Brugada Syndrome: Report of the Brugada Syndrome: Report of the Second Consensus ConferenceSecond Consensus Conference

Appearance of type 3 ST segment elevation Appearance of type 3 ST segment elevation in more than one lead under baseline in more than one lead under baseline conditions with conversion to type 1 after conditions with conversion to type 1 after challenge with a sodium channel blocker is challenge with a sodium channel blocker is considered equivalent to case 1 aboveconsidered equivalent to case 1 above

Drug-induced conversion of type 3 to type 2 Drug-induced conversion of type 3 to type 2 is not considered diagnosticis not considered diagnostic

Characteristic EKG morphologies recorded Characteristic EKG morphologies recorded within the first few hours after resuscitation within the first few hours after resuscitation cannot be taken as diagnosticcannot be taken as diagnostic

Page 71: Sudden Cardiac Arrest  BRUGADA SYNDROME

Drug ChallengeDrug Challenge Drug challenge should be Drug challenge should be

performed while the performed while the patient is continuously patient is continuously monitored and with monitored and with defibrillator and advanced defibrillator and advanced coronary life support coronary life support facilities close at handfacilities close at hand

Drug administration Drug administration should be stopped when should be stopped when the test is positive and/or the test is positive and/or when ventricular when ventricular arrhythmias, including arrhythmias, including ventricular premature ventricular premature complexes, are evident, complexes, are evident, or when significant QRS or when significant QRS widening (30%) is widening (30%) is observed observed

Type 2 and type 3 ECGs, the test is recommended to clarify the diagnosis. Conversion of a type 2 or 3 ECG to a type 1 is considered positive

Proposed Diagnostic Criteria for the Brugada Syndrome: Consensus Report Circulation 2002

Page 72: Sudden Cardiac Arrest  BRUGADA SYNDROME

Differential DiagnosisDifferential Diagnosis Acute myocardial ischemiaAcute myocardial ischemia Acute myocarditisAcute myocarditis Tricyclic antidepressant overdoseTricyclic antidepressant overdose Cocaine intoxicationCocaine intoxication Arrhythmogenic right ventricular Arrhythmogenic right ventricular

dysplasiadysplasia Long QT syndromeLong QT syndrome

Page 73: Sudden Cardiac Arrest  BRUGADA SYNDROME

Arrhythmogenic right ventricular Arrhythmogenic right ventricular dysplasiadysplasia

Discrimination between BrS and ARVC may Discrimination between BrS and ARVC may be particularly difficult because ARVC may be particularly difficult because ARVC may at times mimic BrS at times mimic BrS

Before the diagnosis Brugada syndrome is Before the diagnosis Brugada syndrome is made, a serious attempt should be taken to made, a serious attempt should be taken to exclude ARVC.exclude ARVC.

Drug challenge with sodium channel Drug challenge with sodium channel blockers may be useful in discriminating blockers may be useful in discriminating between these 2 diseasebetween these 2 disease

Page 74: Sudden Cardiac Arrest  BRUGADA SYNDROME

Proposed Diagnostic Criteria for the Brugada Syndrome: Consensus Report Circulation 2002

Page 75: Sudden Cardiac Arrest  BRUGADA SYNDROME

Long QT syndromeLong QT syndrome There is an alteration in the function of a There is an alteration in the function of a

myocellular channel protein that regulates myocellular channel protein that regulates the potassium flux during electrical the potassium flux during electrical repolarizationrepolarization

Clinical course is variable, some remain Clinical course is variable, some remain aymptomatic and some develop syncope aymptomatic and some develop syncope and sudden deathand sudden death

Risk is impacted by factors such as Risk is impacted by factors such as hypokalemia, emotional extremes, and hypokalemia, emotional extremes, and vigorous physical activityvigorous physical activity

Page 76: Sudden Cardiac Arrest  BRUGADA SYNDROME

Long QT syndromeLong QT syndrome involve an abnormal repolarization of involve an abnormal repolarization of

the heart causes differences in the the heart causes differences in the "refractoriness" of the myocytes "refractoriness" of the myocytes leading to re-entrant ventricular leading to re-entrant ventricular arrhythmias arrhythmias

Page 77: Sudden Cardiac Arrest  BRUGADA SYNDROME

Long QT syndromeLong QT syndrome A commonly used criterion to A commonly used criterion to

diagnose LQTS is the LQTS diagnose LQTS is the LQTS "diagnostic score“. Its based on "diagnostic score“. Its based on several criteria giving points to each. several criteria giving points to each. With 4 or more points the probability With 4 or more points the probability is high for LQTS, and with 1 or less is high for LQTS, and with 1 or less point the probability is low. Two or 3 point the probability is low. Two or 3 points indicates intermediate points indicates intermediate probability. probability.

Page 78: Sudden Cardiac Arrest  BRUGADA SYNDROME

Long QT syndromeLong QT syndrome QTc (Defined as QT interval / square root of RR interval) QTc (Defined as QT interval / square root of RR interval)

– >= 480 msec - 3 points >= 480 msec - 3 points – 460-470 msec - 2 points 460-470 msec - 2 points – 450 msec and male gender - 1 point 450 msec and male gender - 1 point

Torsade de Pointes ventricular tachycardia - 2 points Torsade de Pointes ventricular tachycardia - 2 points T wave alternans - 1 point T wave alternans - 1 point Notched T wave in at least 3 leads - 1 point Notched T wave in at least 3 leads - 1 point Low heart rate for age (children) - 0.5 points Low heart rate for age (children) - 0.5 points Syncope (one cannot receive points both for syncope and Torsades Syncope (one cannot receive points both for syncope and Torsades

de pointes) de pointes) – With stress - 2 points With stress - 2 points – Without stress - 1 point Without stress - 1 point

Congenital deafness - 0.5 points Congenital deafness - 0.5 points Family history (the same family member cannot be counted for Family history (the same family member cannot be counted for

LQTS and sudden death) LQTS and sudden death) – Other family members with definite LQTS - 1 point Other family members with definite LQTS - 1 point – Sudden death in immediate family (members before the age 30) - 0.5 Sudden death in immediate family (members before the age 30) - 0.5

points points

Page 79: Sudden Cardiac Arrest  BRUGADA SYNDROME

Long QT syndromeLong QT syndrome

Page 80: Sudden Cardiac Arrest  BRUGADA SYNDROME

Long QT syndromeLong QT syndrome

Beta blockers are the first choice in Beta blockers are the first choice in treating Long QT syndrometreating Long QT syndrome

The only effective form of The only effective form of arrhythmia termination in individuals arrhythmia termination in individuals with LQTS is placement of an with LQTS is placement of an implantable cardioverter-defibrillator implantable cardioverter-defibrillator (ICD). ICD are commonly used in (ICD). ICD are commonly used in patients with syncopes despite beta patients with syncopes despite beta blocker therapy, and in patients who blocker therapy, and in patients who have experienced a cardiac arrest. have experienced a cardiac arrest.

Page 81: Sudden Cardiac Arrest  BRUGADA SYNDROME

Brugada: Risk Brugada: Risk stratificationstratification

Brugada et al found Brugada et al found that patients initially that patients initially presenting with presenting with aborted sudden death aborted sudden death are at the highest risk are at the highest risk for a recurrence for a recurrence (69%)(69%)

Those presenting with Those presenting with syncope and a syncope and a spontaneously spontaneously appearing type 1 ECG appearing type 1 ECG have a recurrence have a recurrence rate of 19%rate of 19%

Men had a 5.5-fold Men had a 5.5-fold higher risk of sudden higher risk of sudden death than did death than did womenwomen

Page 82: Sudden Cardiac Arrest  BRUGADA SYNDROME

Brugada SyndromeBrugada SyndromeIndication for ICD implantationIndication for ICD implantation

Brugada Syndrome Report of the Second Consensus ConferenceEndorsed by the Heart Rhythm Society and the European Heart Rhythm Association

Page 83: Sudden Cardiac Arrest  BRUGADA SYNDROME

Brugada SyndromeBrugada SyndromeIndication for ICD implantationIndication for ICD implantation

Brugada Syndrome Report of the Second Consensus ConferenceEndorsed by the Heart Rhythm Society and the European Heart Rhythm Association

Page 84: Sudden Cardiac Arrest  BRUGADA SYNDROME

Outcome After Implantation of a Outcome After Implantation of a Cardioverter-Defibrillator in Patients With Cardioverter-Defibrillator in Patients With Brugada SyndromeBrugada SyndromeA Multicenter StudyA Multicenter Study Circulation Circulation 20062006

In this large Brugada syndrome In this large Brugada syndrome population, a low incidence of arrhythmic population, a low incidence of arrhythmic events was found, with an annual event events was found, with an annual event rate of 2.6% during a follow-up of 3 yearsrate of 2.6% during a follow-up of 3 years

Device-related complications Device-related complications (8.9%/year). Inappropriate shocks were (8.9%/year). Inappropriate shocks were 2.5 times more frequent than appropriate 2.5 times more frequent than appropriate onesones

Page 85: Sudden Cardiac Arrest  BRUGADA SYNDROME

Efficacy of Quinidine in High-Risk Patients WithEfficacy of Quinidine in High-Risk Patients WithBrugada SyndromeBrugada SyndromeBernard Belhassen, MD; Aharon Glick, MD; Sami Viskin, Bernard Belhassen, MD; Aharon Glick, MD; Sami Viskin, MDMDCirculation Circulation 20042004

Quinidine depresses Quinidine depresses IIto current, which may to current, which may play an important role in the play an important role in the arrhythmogenesis of this diseasearrhythmogenesis of this disease

It prevented VF induction in 22 of the 25 It prevented VF induction in 22 of the 25 patients (88%)patients (88%)

The effective quinidine serum blood levels The effective quinidine serum blood levels rangedfrom 1.29 to 5.2 mg/L (meanSD, rangedfrom 1.29 to 5.2 mg/L (meanSD, 2.650.99 mg/L)2.650.99 mg/L)

Page 86: Sudden Cardiac Arrest  BRUGADA SYNDROME

QuinidineQuinidine May exert its beneficial effects in Brugada May exert its beneficial effects in Brugada

syndrome by inhibiting syndrome by inhibiting IIto, thereby restoring to, thereby restoring electrical homogeneityelectrical homogeneity

In addition, quinidine prolongs ventricular In addition, quinidine prolongs ventricular refractorinessrefractoriness

Finally, the anticholinergic effect of quinidine Finally, the anticholinergic effect of quinidine might contribute to its antiarrhythmic might contribute to its antiarrhythmic efficacy in the Brugada syndromeefficacy in the Brugada syndrome

Despite these hypotheses, the basis for Despite these hypotheses, the basis for quinidine efficacy in this setting remains to quinidine efficacy in this setting remains to be elucidatedbe elucidated

Page 87: Sudden Cardiac Arrest  BRUGADA SYNDROME

Study shows the following:Study shows the following: First, quinidine is highly effective (88% First, quinidine is highly effective (88%

success rate) for preventing VF success rate) for preventing VF induction in patients with inducible VFinduction in patients with inducible VF

Second, it also appears to be effective Second, it also appears to be effective in preventing spontaneous VF, with no in preventing spontaneous VF, with no arrhythmic events observed during a arrhythmic events observed during a mean SD follow-up of 56-67 monthsmean SD follow-up of 56-67 months

Page 88: Sudden Cardiac Arrest  BRUGADA SYNDROME

Third, the drug could be chronically Third, the drug could be chronically tolerated with therapeutic effectiveness tolerated with therapeutic effectiveness in 16 study patients (64%)in 16 study patients (64%)

Fourth, no proarrhythmic event occurred Fourth, no proarrhythmic event occurred in any treated patient despite QT in any treated patient despite QT prolongation. prolongation.

Finally, although quinidine related side Finally, although quinidine related side effects (thrombocytopenia, diarrhea, effects (thrombocytopenia, diarrhea, esophagitis, allergic reaction, aggravation esophagitis, allergic reaction, aggravation of sinus node dysfunction) were common, of sinus node dysfunction) were common, they were always transient and invariably they were always transient and invariably resolved after drug discontinuation resolved after drug discontinuation

Page 89: Sudden Cardiac Arrest  BRUGADA SYNDROME

THANK YOU