Brugada’s Syndrome and Sudden Cardiac Death Petra Lovrec Brugada’s Syndrome and Sudden Cardiac...
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Transcript of Brugada’s Syndrome and Sudden Cardiac Death Petra Lovrec Brugada’s Syndrome and Sudden Cardiac...
Brugada’s Syndrome and Sudden Cardiac Death
Petra Lovrec
Brugada’s Syndrome and Sudden Cardiac Death
Petra LovrecMentor: A. Žmegač Horvat
Sudden Cardiac Death
Incidence
(cases/year)
Survival
Worldwide 3 000 000 ‹1%
U.S. 450 000 5%
W. Europe 400 000 ‹5%
Cardiomyopathy
• dilated cardiomyopathy
• hypertrophic cardiomyopathy
• arrythmogenic right ventricular cardiomyopathy
• left ventricular non-compaction
• restrictive cardiomyopathy
Cardiac Chanellopathies
• Brugada syndrome
• long QT syndrome
• short QT syndrome
• idiopathic VF
• short coupled Torsades
Brugada Syndrome
• first described in 1992 in 8 patients with aborted sudden cardiac death
• history since 1986 – Brugada brothers
Brugada Syndrome
• characterized by:– ECG findings of RBBB and persistent ST
elevation in V1 – V3– structurally normal hearts– propensity for life-threatening ventricular
arrhythmias– J point elevation
Brugada Syndrome: ECG
Typical electrocardiogram of Brugada syndrome. Note the pattern resembling a right bundle branch block,the P-R prolongation and the ST elevation in leads V1-V3.
Brugada Syndrome: ECG
Spontaneous polymorphic ventricular tachycardia recordedduring monitoring in a patient with Brugada syndrome.The arrhythmias are fast and need DC shock to terminate.
Brugada Syndrome: Clinical Features
• unexpectad sudden death
• syncope, seizures
• agonal nocturnal respirations
• affects male patients predominantly (8:1)
• cases reported worldwide
Brugada Syndrome: Incidence and Distribution
• responsible for up to 50% of sudden death victims with structurally normal hearts in Thailand
• recognized in Asia for decades:– Philippines: Bangungut = scream followed by
sudden death during sleep– Thailand: Lai tai = death during sleep– Japan: Pokuri = unexpected death during
sleep
Brugada Syndrome: ECG
12-lead ECG during administration of ajmaline (50 mg in 5 min). Note the progressive appearance of the ST elevation in V1-V3.
Brugada Syndrome: Factors / Drugs that Enhance ECG Pattern
• Na+ channel blockers
• alpha agonists, vagotonic agents, beta blockers
• fever
• alcohol, cocaine
• severe ischemia
• tricyclic antidepressants, antihistaminics
Brugada Syndrome: Genetics
• autosomal dominant• 60 different mutations in the SCN5A
(chromosome 3) gene have been linked to the syndrome
• failure of alpha subunit of sodium channel to express
• shift in voltage and time dependence on I(Na+) activation, inactivation or reactivation
Brugada Syndrome: Diagnosis
• symptoms: syncope, SCD (usually during sleep)• physical exam: normal• family history: strong history of SCD• ECG: best test to identify Brugada patients
– may require Flecainide or Procainamide to bring out typical findings
– ST elevation, RBBB
• imaging tests: usually no underlying structural disease
• stress tests: symptoms and ECG findings not usually reproducible with exercise
Brugada Syndrome: Diagnostic Criteria
• major criteria:– 1. presence of ECG marker in structurally normal
hearts– 2. appearance of ECG marker after administration of
Na+ channel blockers
• minor criteria:– 1. family history of sudden cardiac death– 2. syncope of unknown origin– 3. documented ventricular tachycardia/fibrillation– 4. genetic mutation of ion channels
Brugada Syndrome: Treatment
• IMPLANTABLE CARDIOVERTER-DEFIBRILLATOR (ICD)
• symptomatic with the disease: ICD• asymptomatic with family history of SCD: with normal
ECG – OBSERVE• asymptomatic with prolonged H-V interval and inducible
VT/VF: controversial ICD
Potential Antiarrhythmic Drugs in Brugada Syndrome
• Quinidine (non specific I blocker)
• Isoproterenol (open L type calcium channel)
• Cilostazol (augment I Ca)
Conclusion
• sudden cardiac death in Southeast Asia
• characteristic ECG pattern – ST elevation in V1 – V3
• ICD implantation in symptomatic patients