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. 2018 Dec 4;72(22):2747-2757.
doi: 10.1016/j.jacc.2018.09.037.

Electroanatomic and Pathologic Right Ventricular Outflow Tract Abnormalities in Patients With Brugada Syndrome

Maurizio Pieroni  1 , Pasquale Notarstefano  2 , Antonio Oliva  3 , Oscar Campuzano  4 , Pasquale Santangeli  5 , Monica Coll  6 , Martina Nesti  2 , Andrea Carnevali  7 , Aureliano Fraticelli  2 , Anna Iglesias  6 , Simone Grassi  3 , Ramon Brugada  8 , Leonardo Bolognese  2
Affiliations
Free article

Electroanatomic and Pathologic Right Ventricular Outflow Tract Abnormalities in Patients With Brugada Syndrome

Maurizio Pieroni et al. J Am Coll Cardiol. .
Free article

Abstract

Background: The prevalence and significance of structural abnormalities in Brugada syndrome (BrS) are still largely debated.

Objectives: The authors investigated the relationship between genetic background, electroanatomic abnormalities, and pathologic substrate in BrS.

Methods: They performed 3-dimensional electroanatomic unipolar and bipolar mapping in 30 patients with BrS. Twenty patients underwent 3-dimensional electroanatomic unipolar and bipolar mapping-guided right ventricular outflow tract (RVOT) endomyocardial biopsy. Programmed ventricular stimulation and genetic analysis were performed in all patients.

Results: Low-voltage areas (LVAs) were observed at unipolar map in 93% of patients and at bipolar map in 50% of cases. Unipolar LVAs were always larger than bipolar LVAs, were always colocalized, and in all cases included RVOT. Disease-causing mutations were detected in 10 (33%) patients. Programmed ventricular stimulation was positive in 16 cases (53%). In 75% of patients, RVOT histology showed pathologic findings with myocardial inflammation in 80% of them. Among patients with abnormal bipolar map submitted to endomyocardial biopsy, 9 (81%) showed evidence of myocardial inflammation. Conversely, bipolar map was abnormal in 83% of patients with myocardial inflammation. Myocardial inflammation was also more prevalent among inducible patients (83% vs. 25% in noninducible; p = 0.032).

Conclusions: BrS is characterized by electroanatomical and structural abnormalities localized to RVOT with a gradient of the pathologic substrate from epicardium to endocardium possibly driven by myocardial inflammation. These findings reclassify BrS as a combination of structural and electrical defects opening the way to new risk stratification and therapeutic strategies.

Keywords: Brugada syndrome; electroanatomic mapping; endomyocardial biopsy; genetic analysis; myocardial inflammation; sudden cardiac death.

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Comment in

  • Brugada Syndrome: In Search of a Cause.
    Corrado D, Migliore F, Zorzi A. Corrado D, et al. J Am Coll Cardiol. 2018 Dec 4;72(22):2758-2760. doi: 10.1016/j.jacc.2018.08.2199. J Am Coll Cardiol. 2018. PMID: 30497562 No abstract available.
  • Myocardial Inflammation in Brugada Syndrome.
    Miles C, Asimaki A, Behr ER, Sheppard MN. Miles C, et al. J Am Coll Cardiol. 2019 Mar 26;73(11):1369-1370. doi: 10.1016/j.jacc.2018.12.058. J Am Coll Cardiol. 2019. PMID: 30898219 No abstract available.
  • Reply: Myocardial Inflammation in Brugada Syndrome.
    Pieroni M, Notarstefano P, Oliva A, Santangeli P, Bolognese L. Pieroni M, et al. J Am Coll Cardiol. 2019 Mar 26;73(11):1370. doi: 10.1016/j.jacc.2019.01.023. J Am Coll Cardiol. 2019. PMID: 30898220 No abstract available.

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