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Low rate of life-threatening events and limitations in predicting invasive and non-invasive markers of symptoms in a cohort of type 1 Brugada syndrome patients. Data and insights from the GenBra Registry.
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  • Luciana Sacilotto,
  • Mauricio Scanavacca,
  • Natália Olivetti,
  • Carolina Lemes,
  • Gabrielle PessenteOrcid,
  • Fanny Wulkan,
  • Denise Hachul,
  • José Eduardo Krieger,
  • Alexandre Pereira,
  • Francisco DarrieuxOrcid
Luciana Sacilotto
Universidade de Sao Paulo Faculdade de Medicina Hospital das Clinicas Instituto do Coracao
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Mauricio Scanavacca
Universidade de Sao Paulo Faculdade de Medicina Hospital das Clinicas Instituto do Coracao
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Natália Olivetti
Universidade de Sao Paulo Faculdade de Medicina Hospital das Clinicas Instituto do Coracao
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Carolina Lemes
Universidade de Sao Paulo Faculdade de Medicina Hospital das Clinicas Instituto do Coracao
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Gabrielle Pessente
Orcid
Universidade de Sao Paulo Faculdade de Medicina Hospital das Clinicas Instituto do Coracao
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Fanny Wulkan
Universidade de Sao Paulo Faculdade de Medicina Hospital das Clinicas Instituto do Coracao
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Denise Hachul
Universidade de Sao Paulo Faculdade de Medicina Hospital das Clinicas Instituto do Coracao
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José Eduardo Krieger
Universidade de Sao Paulo Faculdade de Medicina Hospital das Clinicas Instituto do Coracao
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Alexandre Pereira
Universidade de Sao Paulo Faculdade de Medicina Hospital das Clinicas Instituto do Coracao
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Francisco Darrieux
Orcid
Universidade de Sao Paulo Faculdade de Medicina Hospital das Clinicas Instituto do Coracao
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Peer review status:UNDER REVIEW

12 Jun 2020Submitted to Journal of Cardiovascular Electrophysiology
13 Jun 2020Assigned to Editor
13 Jun 2020Submission Checks Completed
14 Jun 2020Reviewer(s) Assigned

Abstract

Background: Brugada syndrome (BrS) has diagnostic challenges and controversial risk assessment. We aimed to investigate invasive and non-invasive parameters in symptomatic and asymptomatic patients from a Brazilian cohort of type-1 BrS. Methods Patients with spontaneous and drug-induced type-1 BrS were classified in two groups, asymptomatic (n=116, 84.1%) and symptomatic (n=22, 15.9%, 13 with arrhythmogenic syncope, 9 with aborted sudden cardiac death). Genetic testing, EPS parameters, and ECG parameters were analysed. Results: 138 consecutive patients were eligible, 101 men (73.2%), mean 41.4 years, mostly probands (79%). Spontaneous pattern, observed in 77.5% of the patients, was associated to symptoms only if expressed in V1 and V2 standard position (not high precordial leads) (p=0.014). All symptomatic patients were probands. The presence of RV outflow tract conduction delay (RVOTcd) signs, positive EPS and SCN5A status was similar between symptomatic and asymptomatic subjects. During mean 75-month follow-up, 8 patients had appropriate therapies. All had spontaneous type-1 ECG pattern and 2/8 (25%) were asymptomatic, with positive EPS. The overall LAE incidence of 1.1%/year dropped to 0.27% in asymptomatic patients. RVOTcd occurred more frequently in SCN5A carriers (QRS-f 33.3% vs 7.7%, p=0.005; AVR sign 58.3% vs 13.6%, p<0.001; deep S in lead I 75% vs 48.5%, p=0.025%), as well as longer HV interval (66ms vs 49ms, p<0.001). Conclusions: Spontaneous type-1 Brugada pattern in standard leads and proband status were more frequent in symptomatic subjects. RVOTcd, more common in SCN5A carriers, did not predict symptoms in BrS patients. EPS exhibited limited prognostic value for this low risk population.