Download - Brugada case-presentation
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Rare case diagnosed in El Ansar hospital
The first time to see This
Rare SYNDROME
* 40 years old male patient, smoker, no HTN, no DM.
*Two years ago he had fainting attack, non typical chest pain.
*Misdiagnosed in Yemen as Cardiac Ischemia,,, Again 1 year ago misdiagnosed in Saudi Arabia.
*He was admitted to our hospital with acute pneumonia.
* Routine ECG was done and it was amazing (ST elevation!!!).
Brugada Syndrome
• Discovered by the Brugada brothers in 1992.
• Inherited defect of sodium channels (SCN5A gene).
• Epicardial area of the RV has repolarization abnormality.
• Prone to spontaneous Ventricular Arrhythmias.
Types
• Three ECG patterns of pseudo RBBB (incomplete or complete) with ST elevation in V1, V2, V3.
• Type I is diagnostic, but type II, III are suspicious and can be converted into type I with NA channel blocker (Ajmalin challenge test) or Fever or vagotonic stimulation.
Pathogenesis
• Genetics (Autosomal dominant)
• Loss of function mutations in SCN5A(3p21-24) coding for Alpha subunit of cardiac sodium channel
• The defective myocardial sodium channels reduce sodium inflow currents, thereby reducing the duration of normal action potentials.
• In addition, a prominent transient outward current, called I(to), in the right ventricular epicardium further shortens the action potential.
• This decrease in INa is thought to affect the right ventricular endocardium differently from the epicardium causing a voltage gradient during repolarization and the characteristic ST elevations on ECG.
Signs and symptoms*Syncope and cardiac arrest (due to ventricular arrhythmias: VT, VF): Most common clinical manifestations; in many cases, cardiac arrest occurs during sleep or rest, nightmares at night.*Asymptomatic, but routine ECG shows ST-segment elevation in leads V1-V3.
*Associated atrial fibrillation (20%).
*Fever: Often reported to trigger or exacerbate clinical manifestations, ECG appearance.
Diagnosis
*Most patients with Brugada syndrome have a normal physical examination. *12-lead ECG in all patients with syncope*Drug challenge with a sodium channel. blocker in patients with syncope without an obvious cause.
Management• Preventing sudden death(VT, VF) in
patients with Brugada syndrome through implantation of an automatic implantable
cardiac defibrillator (ICD).
* No pharmalogical treatment is
effective.
Don’t give Brugada patients Na+ Channel Blockers!
• TCAs
• Flecinide
• Procainamide
• Bupivicaine
• Lidocaine
• Amiodarone
Prognosis
• When a patient begins showing signs of Brugada Syndrome (syncope), mortality increases by 10% for every year Brugada is untreated.
• *FEVER gave hand to us to diagnose him as it elicited the ECG appearance of BRUGADA (Converting it from type III into type I).
Sudden Cardiac Death in YoungCoronary artery anomalies, LAD bridge, CADPrimary electrical diseases:
WPW Long QT syndrome Catecholaminergic polymorphic VT Brugada syndrome Short QT syndrome Idiopathic VFNeurologic problems