brugada case-presentation

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Case Presentation By Islam Ghanem Cardiologist

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Case PresentationBy

Islam GhanemCardiologist

Think For A minute, You may Save A life

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

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.

Brugada = Sailboat of DEATH in V1-V3.

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.

Ventricular Tachycardia

Ventricular Fibrillation

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.

*Echocardiography.

*CK-MB and troponin levelsGenetic testing for a mutation in SCN5A.

Typical Brugada EKG

Brugada Syndrome leads to ventricular arrhythmias.

Post cardiac arrest 41 y/o

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.

Back to our patient

• *FEVER gave hand to us to diagnose him as it elicited the ECG appearance of BRUGADA (Converting it from type III into type I).

The patient was sent to a higher centre for ICD implantation

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

T h a n k y o u