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1. CASE PRESENTATION
INTERNAL MEDICINE
Resident: Pham Tuan Nghia Faculty: Nguyen Nam Duong Teaching resident: Nguyen Dinh Tung
14 May 2024

2. CHIEF COMPLAINT
46-year-old female patient diagnosed with complicated UTI,
hospitalized day 2, being treated with tazocin on day 1.
PMH revealed:
2 syncope episodes, 3 and 1 years ago;
1 episode of lightheadedness caused by severe palpitation a couple of
months ago.

3. Present illness
The patient was alert, oriented.
Mild headache and muscle ache.
No rhinorrhea, no cough, no sore throat, no SOB, no chest pain, no
abdominal pain, no dysuria.

4. DISCUSSION
 What additional information would you like to obtain from her?

5. The syncope episodes characteristics
History of syncope: 2 episodes, 3 years and 1 year ago
Triggers: Could not recall
Symptoms before fainting: Dizziness and lightheadedness
Location of fainting episodes: Workplace
No witnesses to fainting episodes
During the syncope episodes: No convulsions, no perception of
sound or image.
No urinary incontinence, no sweating, anxiety.
Duration of fainting episodes: Approximately 30 minutes
No post-ictal phase, but she feel weak after the episodes.

6. The pre-syncope episode characteristics
She experienced severe palpitation
Sweating
Sense of impending doom
“Everything started to turn black”
Loss of all the strength and had to gasp for air.

7. Past medical history
Other PMH:
Gastritis
 Asymptomatic Adenomyosis.
Family history: Mother and Uncle passed away suddenly at 48 and
52 years old when sleeping, respectively.
Medication:
Surgical history: None
Allergy history: None
Social history: No drinking, no smoking.
ObGyn: normal menstruation.

8. PHYSICAL EXAMINATION
 VS: BP: 120/72 mmHg Temp: 38.5o C
RR: 19/minute HR: 84/minute
 General: Alert and Oriented, no palpable lymphnodes
 CV: S1/S2 clear, no murmurs, rubs, or gallops
 Lungs: Clear To Auscultation Bilaterally, no rales/wheeze/rhonchi
 Abdomen: soft nontender, Bowel Sound (+),
Hepatosplenomegaly(-)
 Extremities: No abnormal findings
 Skin: No abnormal findings
 Neuro: No abnormal findings

9. Next step?
• What would you do next?

10. LABORATORY TEST
1. Hematology
2. Biochemistry
3. Urinanalysis
Hematology
RBC HGB HCT MCV WBC NEU PLT
3.77 T/L 106 g/L 0.327 % 86.6 fL 12.6 G/l 88.0% 154 G/L
Creatinin CRP AST ALT Troponin T Na+/K+/Cl-
57 umol/L 71.9 umol/L 19 U/L 11 U/L 3 ng/L (<14) 137/3.26/103
mmol/L
Leucocyte Protein Nitrate albumin creatinine Culture
70 negative negative 10 4.4 Negative.

11. LABORATORY TEST
1. Hematology
2. Biochemistry
3. Urinanalysis
Hematology
RBC HGB HCT MCV WBC NEU PLT
3.77 T/L 106 g/L 0.327 % 86.6 fL 12.6 G/l 88.0% 154 G/L
Creatinin CRP AST ALT Troponin T Na+/K+/Cl-
57 umol/L 71.9 umol/L 19 U/L 11 U/L 3 ng/L (<14) 137/3.26/103
mmol/L
Leucocyte Protein Nitrate albumin creatinine Culture
70 negative negative 10 4.4 Negative.

12. Next step?

13. Next step?

14. Brugada pattern
ECG - AMBOSS
– ST elevation ≥ 2 mm and a
negative T wave in V1 and
V2 (red overlay). The shape
of ST elevation seen in V1 is
described as “coved.”
– Pseudo-RBBB: The ST
changes create a pattern
resembling RBBB in V1.
Pseudo-RBBB with ST
elevation in V1–V3 is
characteristic of Brugada
pattern. To diagnose
Brugada syndrome, the
corresponding clinical
criteria must also be met,
e.g., VF, syncope, or
pertinent family history.

15. ECG patterns of Brugada syndrome in leads
V1-V2
(A) This typical coved pattern present in V1-V2 shows the following:
At the end of QRS, an ascending and quick slope with a high take-off ≥2 mm followed
by concave or rectilinear downsloping ST. There are few cases of coved pattern with a high take-off
between 1 and 2 mm.
1. There is no clear r' wave.
2. The high take-off often does not correspond with the J point.
3. At 40 milliseconds of high take-off, the decrease in amplitude of ST is ≤4 mm
4. ST at high take-off N ST at 40 milliseconds N ST at 80 milliseconds.
5. ST is followed by negative and symmetric T wave.
6. The duration of QRS is longer than in RBBB, and there is a mismatch between V1 and V6.
(B) This typical saddle-back pattern present in V1-V2 shows the following:
High take-off of r' (that often does not coincide with J point) ≥2 mm.
1. Descending arm of r' coincides with beginning of ST (often is not well seen).
2. Minimum ST ascent ≥0.5 mm.
3. ST is followed by positive T wave in V2 (T peak N ST minimum N 0) and of variable morphology
in V1.
4. The characteristics of triangle formed by r' allow to define different criteria useful for diagnosis.
1. β angle.
2. Duration of the base of the triangle of r' at 5 mm from the high take-off greater than 3.5
mm.
Brugada syndrome: Clinical presentation, diagnosis, and evaluation – UpToDate
Management of patients with a Brugada ECG pattern (escardio.org)
coved pattern saddle-back pattern

16. BRUGADA PATTERN VERSUS SYNDROME?
Brugada pattern: typical ECG features AND asymptomatic + have no
other clinical criteria
Brugada syndrome: typical ECG features AND experience of sudden
cardiac death + one or more of the other associated clinical criteria.
What are they?

17. Associated clinical criteria
Sudden cardiac arrest resulting from ventricular tachyarrhythmia
Sudden unexpected noctural death syndrome
More common at night
occur more commonly during sleep
are not usually secondary to exercise
Syncope
ventricular arrhythmia or nonarrhythmic causes (eg, neurocardiogenic)
Palpitations
atrial fibrillation

18. ONE LINER CHALLENGE
 Can you summarize the problems of this patient in one sentence?

19. One-liner
• 46-year-old female patient with a PMH of unknown-cause syncope,
family history of sudden death, presents with Brugada pattern in V1
and V2, which suggests Brugada syndrome.
Horizontal Nystagmus

20. Management?
 What is the next step in management?
Horizontal Nystagmus

21. ACUTE TREATMENT
Inform relatives of the sudden cardiac death event, especially when
the patient in fever episode.
Manage fever
Avoiding specific medications (Class I antiarrhythmic drugs;
psychotropic drugs – Tricyclic antidepressants, lithium, and
oxcarbazepine; anesthesia medications – Procaine, bupivacaine and
prolonged propofol infusion )
Correct metabolite disbalance
Testing for underlying heart disease (echo, cardiac stress testing,
MRI)

22. Manage fever? Why?
Brugada pattern is more common in patients with fever – In a study of
402 febrile emergency department patients and 909 controls, type I Brugada
pattern ECG changes were 20 times more common in febrile patients (2 versus
0.1 percent. Reassuringly, none of these patients had cardiac events over 30
months of follow-up.
Sudden cardiac arrest in febrile patients with Brugada syndrome – In a single-
center retrospective review of 111 patients with confirmed Brugada syndrome,
22 patients had cardiac arrest, of whom four (18 percent) had a preceding fever.
In a subset of 24 of the 111 patients with ECGs recorded
during fever and normothermia, ECGs taken during fever had
prolonged QRS and QT intervals and worsening ST elevation

23. FOLLOW-UP TREATMENT PLAN
Would you recommend implantable cardiac defibrillator for this
patient?
Implantable cardioverter-defibrillators (ICDs) - Mayo Clinic

24. RISK ASSESSMENT FOR ARRHYTHMIA OR
SUDDEN CARDIAC ARREST
• High-risk symptoms: Brugada pattern +
• Sudden cardiac arrest,
• Syncope (unexplained syncope suggestive of a tachyarrhythmia),
• Sustained ventricular tachycardia,
• Nocturnal agonal respiration
• Intermediate-risk factors:
• Syncope that may be nonarrhythmic in origin
• Family history of SCA and/or Brugada Syndrome
• Drug-induced type 1 ECG pattern
• Atrial fibrillation (AF)
Brugada syndrome or pattern: Management and approach to screening of relatives - UpToDate

25. Implantable cardioverter defibrillator (ICD)
Consensus recommendations for
implantable cardioverter-defibrillators
(ICDs) in patients diagnosed with
Brugada syndrome
ECG: electrocardiogram; EP: electrophysiology; ICD:
implantable cardioverter-defibrillator; SCD: sudden cardiac
death; VF: ventricular fibrillation; VT: ventricular
tachycardia
Brugada syndrome or pattern: Management and approach to screening of relatives - UpToDate

26. Clinical Approach to Brugada Syndrome
• ECG: electrocardiogram; ICD: implantable cardioverter-
defibrillator; VT: ventricular
tachycardia; NSAID: nonsteroidal anti-inflammatory drug.
• * Acetaminophen preferred; NSAID may be added if
needed in absence of risk factors for NSAID toxicity.
• Δ High-risk features include sudden cardiac
arrest, arrhythmogenic syncope, documented sustained VT,
and family history of Brugada syndrome.
• § Quinidine oral dose is 1 to 1.5 g/day of quinidine sulfate
or 600 to 900 mg/day of hydroquinidine (not available in
the United States), typically divided into three to four equal
daily doses. Small studies have suggested that lower doses
of quinidine sulfate (300 to 600 mg/day) may be effective
in some patients. Strength of quinidine products available
outside the United States may be expressed as salt
or quinidine base; refer to local labeling for equivalence
before use.
• ¥ Amiodarone oral dose is 200 mg daily after an initial
oral loading dose (typically 400 mg twice daily or 200 mg
three times daily for one to two weeks).
• ‡ Significant burden of sustained ventricular tachycardia.
Brugada syndrome or pattern: Management and approach to screening of relatives - UpToDate

27. FOLLOW UP
C L I N I C A L E L E C T R O P H Y S I O L O G Y V O L . 8 , N O . 3 , 2 0 2 2 Krahn et al

28. • CPVT: catecholaminergic polymorphic
ventricular tachycardia
• LQT: long QT syndrome
• BrS, Brugada syndrome
• ACS, acute coronary syndrome
• HCM, hypertrophic cardiomyopathy
• DCM, dilated cardiomyopathy
• ARVC, arrhythmogenic right ventricular
cardiomyopathy
• rTOF, repaired tetralogy of Fallot
• PVT, polymorphic ventricular
tachycardia
• MVT, monomorphic ventricular tachycardia
2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death | European Heart Journal | Oxford Academic (oup.com)

29. FOLLOW UP
• Educate the patient and relatives about the nature of the disease,
how to recognize the symptoms and avoid provoking factors.
• Screening relatives (first-degree relatives)
• Clinical symptoms
• ECG
• Positive results → risk stratification
• Indeterminate results → should undergo drug-challenge testing.
• Negative results
• no history of syncope + a normal ECG → negative screening result
• With clinical symptom + normal ECG → repeat every one to two years until at least the fifth
decade of life

30. Genetic testing?
• Brugada syndrome Definition: The Brugada syndrome is
an autosomal dominant genetic disorder with variable expression
characterized by abnormal findings on the
surface electrocardiogram (ECG) in conjunction with an increased
risk of ventricular tachyarrhythmias and sudden cardiac death.
• Mutations in SCN5A, SCN10A, which encode
• subunits of sodium channels in the
• epicardial, endocardial and M cells of ventricular myocardium,
• which leads to the decrease of action potential duration (Na channel – depolarization phase)
and the relative increase in the transient outward current (K channel – repolarization phase).
• Hyperthermia causes changes in sodium current function; a
reduction in sodium current results in changes to the action potential
predisposing to ventricular fibrillation.

31. Genetic testing?
1.Perform genetic testing for the proband (the affected patient) with Brugada
syndrome.
2.If a pathogenic variant is identified in the proband, then test their first-degree
relatives (parents, siblings, children) for the specific variant found in the
proband. This is called targeted genetic testing.
3.However, universal genetic testing of asymptomatic first-degree relatives is not
recommended, even if the proband has a confirmed diagnosis of Brugada
syndrome but no pathogenic variant is identified.
• The rationale provided is that evidence does not support universal genetic
testing of asymptomatic relatives, as the presence of potentially pathogenic
variants in SCN5A or KCNH2 genes did not significantly increase the risk of
being diagnosed with an arrhythmia syndrome in the study cited.

32. What is the chance of seeing this pattern and
syndrome again? - Epidemiology
• Prevalence of Brugada pattern: 0.1 and 1 percent based on the studied
populations, Brugada syndrome is significantly lower.
• Male > female
• Age at diagnosis - diagnosed in adulthood - the average patient age was 41
years (+-15).

33. Key takeaways

34. Key takeaways
1. Thorough, systematic history taking is critical.
2. Recognize the Brugada pattern on ecg
3. Risk stratify patients with Brugada pattern.
4. Acute management plan and follow up.