Real case of Brugada syndrome in KSA by Islam Ghanem -Assistant lecturer of cardiology-Zagazig university-Egypt.

1. Case Presentation
By
Islam Ghanem
Cardiologist

2. Think For A minute, You may
Save A life
Rare case diagnosed in El
Ansar hospital
The first time to see This
Rare SYNDROME

4. * 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.

5. *He was admitted to our hospital with
acute pneumonia.
* Routine ECG was done and it was
amazing (ST elevation!!!).

7. Brugada Syndrome

8. 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.

9. Brugada = Sailboat of DEATH in
V1-V3.

10. 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.

13. 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.

14. • 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.

15. 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.

16. *Associated atrial fibrillation (20%).
*Fever: Often reported to trigger or
exacerbate clinical manifestations, ECG
appearance.

17. Ventricular Tachycardia

18. Ventricular Fibrillation

20. 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.

21. *Echocardiography.
*CK-MB and troponin levels
Genetic testing for a mutation in SCN5A.

22. Typical Brugada EKG

23. Brugada Syndrome leads to
ventricular arrhythmias.

26. Post cardiac arrest 41 y/o

27. Management
• Preventing sudden death(VT, VF) in
patients with Brugada syndrome through
implantation of an automatic implantable
cardiac defibrillator (ICD).
* Nopharmalogical treatment is
effective.

30. Don’t give Brugada patients Na+
Channel Blockers!
• TCAs
• Flecinide
• Procainamide
• Bupivicaine
• Lidocaine
• Amiodarone

31. Prognosis
• When a patient begins showing signs of
Brugada Syndrome (syncope), mortality
increases by 10% for every year Brugada
is untreated.

32. Back to our patient

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

41. The patient was sent to
a higher centre for ICD
implantation

42. Sudden Cardiac Death in Young
Coronary artery anomalies, LAD bridge, CAD
Primary electrical diseases:
 WPW
 Long QT syndrome
 Catecholaminergic polymorphic VT
 Brugada syndrome
 Short QT syndrome
 Idiopathic VF
Neurologic problems

44. Thank
you