Brugada syndrome - where do we stand today - case reports

    Authors

    Keywords

    Brugada syndrome, sudden cardiac death, risk stratification

    DOI

    https://doi.org/10.15836/ccar2016.425

    Full Text

    **Introduction:** Brugada syndrome (BrS) has low prevalence (14-30:10000), it is more common in men and people from Asia, and it is characterized by typical ECG changes as well as high incidence of sudden cardiac death (SCD). ICD implantation is the only SCD prevention option. HRS/EHRA/APHRS Consensus article (1) states the indications for ICD implantation, but asymptomatic patients, or those with syncope of unclear origin can be hard for risk stratification. (2, 3) **Case reports:** We are presenting two cases from our hospital. A 40-year-old male was seen for atrial fibrillation and type II ECG changes. There were no SCD in family, and he never had syncope. Pulmonary vein isolation (PVI) was done, then ajmaline test (positive) and finally electrophysiology study (EPS) (negative). A 36-year-old male with type II ECG was admitted after syncope which occurred during rest on a hot day at a picnic. Holter as well as neurologic workup were normal. Ajmaline test was positive and EPS negative. Both patients were instructed to avoid specific drugs, to change lifestyle and prevent high fever with antipyretics. Guidelines clearly state ICD should be implanted to SCD survivors or sustained ventricular tachycardia (VT), it can be useful in patients with type I ECG changes and a syncope, and it may be considered in EPS inducible patients. First patient is in follow up because he doesn’t fulfill any criteria. Also he was previously treated with propafenone without any problems, which is also a possible sign of lower risk for SCD, finally he was successfully treated for atrial fibrillation, which is linked ventricular arrhythmias in BrS patients, so this was also a tool to lower his risk. Second patients had a syncope, but other syncope etiologies are possible, he has type II ECG changes and arrhythmia is not inducible therefore he is also in follow up. **Conclusion:** Potentially lethal arrhythmias in asymptomatic patients develop in 1-8% in 3 years (Brugada; Eckardt; Probst). Finally, EPS reccommendations are not clearely defined, therefore we are still conducting EPS as an adjuvant tool for risk stratification.

    Literature

    1. Priori SG, Wilde AA, Horie M, Cho Y, Behr ER, Berul C, et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes: document endorsed by HRS, EHRA, and APHRS in May 2013 and by ACCF, AHA, PACES, and AEPC in June 2013. Heart Rhythm. 2013;10(12):1932–63. https://doi.org/10.1016/j.hrthm.2013.05.014
    2. Eckardt L, Probst V, Smits JP, Bahr ES, Wolpert C, Schimpf R, et al. Long-term prognosis of individuals with right precordial ST-segment-elevation Brugada syndrome. Circulation. 2005;111(3):257–63. https://doi.org/10.1161/01.CIR.0000153267.21278.8D
    3. Brugada J, Brugada R, Brugada P. Determinants of sudden cardiac death in individuals with the electrocardiographic pattern of Brugada syndrome and no previous cardiac arrest. Circulation. 2003;108:3092–6. https://doi.org/10.1161/01.CIR.0000104568.13957.4F
    Cardiologia Croatica
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    Brugada syndrome - where do we stand today - case reports

    Extended Abstract
    Issue10-11
    Published
    Pages425
    PDF via DOIhttps://doi.org/10.15836/ccar2016.425
    Brugada syndrome
    sudden cardiac death
    risk stratification

    Authors

    Matija Marković*ORCIDClinical Hospital Merkur, Zagreb, Croatia
    Ena KurtićORCIDClinical Hospital Merkur, Zagreb, Croatia
    Darko PočanićORCIDClinical Hospital Merkur, Zagreb, Croatia
    Mario StipinovićORCIDClinical Hospital Merkur, Zagreb, Croatia
    Stjepan KranjčevićORCIDClinical Hospital Merkur, Zagreb, Croatia
    Helena JerkićORCIDClinical Hospital Merkur, Zagreb, Croatia
    Tomislav LetilovićORCIDClinical Hospital Merkur, Zagreb, Croatia
    Damir KozmarORCIDClinical Hospital Merkur, Zagreb, Croatia
    Maro DragičevićORCIDClinical Hospital Merkur, Zagreb, Croatia
    Ivica Premužić MeštrovićORCIDClinical Hospital Merkur, Zagreb, Croatia

    *Correspondence email: mtj.markovic@gmail.com

    Full Text

    Introduction: Brugada syndrome (BrS) has low prevalence (14-30:10000), it is more common in men and people from Asia, and it is characterized by typical ECG changes as well as high incidence of sudden cardiac death (SCD). ICD implantation is the only SCD prevention option. HRS/EHRA/APHRS Consensus article (1) states the indications for ICD implantation, but asymptomatic patients, or those with syncope of unclear origin can be hard for risk stratification. (2, 3)

    Case reports: We are presenting two cases from our hospital. A 40-year-old male was seen for atrial fibrillation and type II ECG changes. There were no SCD in family, and he never had syncope. Pulmonary vein isolation (PVI) was done, then ajmaline test (positive) and finally electrophysiology study (EPS) (negative). A 36-year-old male with type II ECG was admitted after syncope which occurred during rest on a hot day at a picnic. Holter as well as neurologic workup were normal. Ajmaline test was positive and EPS negative. Both patients were instructed to avoid specific drugs, to change lifestyle and prevent high fever with antipyretics. Guidelines clearly state ICD should be implanted to SCD survivors or sustained ventricular tachycardia (VT), it can be useful in patients with type I ECG changes and a syncope, and it may be considered in EPS inducible patients. First patient is in follow up because he doesn’t fulfill any criteria. Also he was previously treated with propafenone without any problems, which is also a possible sign of lower risk for SCD, finally he was successfully treated for atrial fibrillation, which is linked ventricular arrhythmias in BrS patients, so this was also a tool to lower his risk. Second patients had a syncope, but other syncope etiologies are possible, he has type II ECG changes and arrhythmia is not inducible therefore he is also in follow up.

    Conclusion: Potentially lethal arrhythmias in asymptomatic patients develop in 1-8% in 3 years (Brugada; Eckardt; Probst). Finally, EPS reccommendations are not clearely defined, therefore we are still conducting EPS as an adjuvant tool for risk stratification.

    Literature

    1. 1.
      Priori SG, Wilde AA, Horie M, Cho Y, Behr ER, Berul C, et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes: document endorsed by HRS, EHRA, and APHRS in May 2013 and by ACCF, AHA, PACES, and AEPC in June 2013. Heart Rhythm. 2013;10(12):1932–63.DOI
    2. 2.
      Eckardt L, Probst V, Smits JP, Bahr ES, Wolpert C, Schimpf R, et al. Long-term prognosis of individuals with right precordial ST-segment-elevation Brugada syndrome. Circulation. 2005;111(3):257–63.DOI
    3. 3.
      Brugada J, Brugada R, Brugada P. Determinants of sudden cardiac death in individuals with the electrocardiographic pattern of Brugada syndrome and no previous cardiac arrest. Circulation. 2003;108:3092–6.DOI