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Long-term prognosis of women with Brugada syndrome and electrophysiological study

Published:December 24, 2020DOI:https://doi.org/10.1016/j.hrthm.2020.12.020

      Background

      A male predominance in Brugada syndrome (BrS) has been widely reported, but scarce information on female patients with BrS is available.

      Objective

      The purpose of this study was to investigate the clinical characteristics and long-term prognosis of women with BrS.

      Methods

      A multicenter retrospective study of patients diagnosed with BrS and previous electrophysiological study (EPS) was performed.

      Results

      Among 770 patients, 177 (23%) were female. At presentation, 150 (84.7%) were asymptomatic. Females presented less frequently with a type 1 electrocardiographic pattern (30.5% vs 55.0%; P <.001), had a higher rate of family history of sudden cardiac death (49.7% vs 29.8%; P <.001), and had less sustained ventricular arrhythmias (VAs) on EPS (8.5% vs 15.1%; P = .009). Genetic testing was performed in 79 females (45% of the sample) and was positive in 34 (19%). An implantable cardioverter-defibrillator was inserted in 48 females (27.1%). During mean (± SD) follow-up of 122.17 ± 57.28 months, 5 females (2.8%) experienced a cardiovascular event compared to 42 males (7.1%; P = .04). On multivariable analysis, a positive genetic test (18.71; 95% confidence interval [CI] 1.82–192.53; P = .01) and atrial fibrillation (odds ratio 21.12; 95% CI 1.27–350.85; P = .03) were predictive of arrhythmic events, whereas VAs on EPS (neither with 1 or 2 extrastimuli nor 3 extrastimuli) were not.

      Conclusion

      Women with BrS represent a minor fraction among patients with BrS, and although their rate of events is low, they do not constitute a risk-free group. Neither clinical risk factors nor EPS predicts future arrhythmic events. Only atrial fibrillation and positive genetic test were identified as risk factors for future arrhythmic events.

      Keywords

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      References

        • Brugada P.
        • Brugada J.
        Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. A multicenter report.
        J Am Coll Cardiol. 1992; 20: 1391-1396
        • Sieira J.
        • Conte G.
        • Ciconte G.
        • et al.
        Clinical characterisation and long-term prognosis of women with Brugada syndrome.
        Heart. 2016; 102: 452-458
        • Benito B.
        • Sarkozy A.
        • Mont L.
        • et al.
        Gender differences in clinical manifestations of Brugada syndrome.
        J Am Coll Cardiol. 2008; 52: 1567-1573
        • Di Diego J.M.
        • Cordeiro J.M.
        • Goodrow R.J.
        • et al.
        Ionic and cellular basis for the predominance of the Brugada syndrome phenotype in males.
        Circulation. 2002; 106: 2004-2011
        • Barajas-Martinez H.
        • Haufe V.
        • Chamberland C.
        • et al.
        Larger dispersion of INa in female dog ventricle as a mechanism for gender-specific incidence of cardiac arrhythmias.
        Cardiovasc Res. 2009; 81: 82-89
        • Song M.
        • Helguera G.
        • Eghbali M.
        • et al.
        Remodeling of Kv4.3 potassium channel gene expression under the control of sex hormones.
        J Biol Chem. 2001; 276: 31883-31890
        • Rodríguez-Mañero M.
        • Casado-Arroyo R.
        • Sarkozy A.
        • et al.
        The clinical significance of pregnancy in Brugada syndrome.
        Rev Esp Cardiol. 2014; 67: 176-180
        • Antzelevitch C.
        • Brugada P.
        • Borggrefe M.
        • et al.
        Brugada syndrome: report of the second consensus conference: endorsed by the Heart Rhythm Society and the European Heart Rhythm Association.
        Circulation. 2005; 111: 659-670
        • Priori S.G.
        • Wilde A.A.
        • Horie M.
        • et al.
        Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes.
        Europace. 2013; 15: 1389-1406
        • Dereci A.
        • Yap S.C.
        • Schinkel A.F.L.
        • et al.
        Meta-analysis of clinical outcome after implantable cardioverter-defibrillator implantation in patients with Brugada syndrome.
        JACC Clin Electrophysiol. 2019; 5: 141-148
        • Probst V.
        • Veltmann C.
        • Eckardt L.
        • et al.
        Long-term prognosis of patients diagnosed with Brugada syndrome: results from the FINGER Brugada Syndrome Registry.
        Circulation. 2010; 121: 635-643
        • Priori S.G.
        • Gasparini M.
        • Napolitano C.
        • et al.
        Risk stratification in Brugada syndrome: results of the PRELUDE (PRogrammed ELectrical stimUlation preDictive valuE) registry.
        J Am Coll Cardiol. 2012; 59: 37-45
        • Pablo-Flórez J.
        • García D.
        • Valverde I.
        • et al.
        Role of syncope in predicting adverse outcomes in patients with suspected Brugada syndrome undergoing standardized flecainide testing.
        Europace. 2018; 20: 64-71
        • Sarkozy A.
        • Boussy T.
        • Kourgiannides G.
        • et al.
        Long-term follow-up of primary prophylactic implantable cardioverter-defibrillator therapy in Brugada syndrome.
        Eur Heart J. 2007; 28: 334-344
        • Makimoto H.
        • Kamakura S.
        • Aihara N.
        • et al.
        Clinical impact of the number of extrastimuli in programmed electrical stimulation in patients with Brugada type 1 electrocardiogram.
        Heart Rhythm. 2012; 9: 242-248
        • Letsas K.P.
        • Liu T.
        • Shao Q.
        • et al.
        Meta-analysis on risk stratification of asymptomatic individuals with the Brugada phenotype.
        Am J Cardiol. 2015; 116: 98-103
        • Sroubek J.
        • Probst V.
        • Mazzanti A.
        • et al.
        Programmed ventricular stimulation for risk stratification in the Brugada syndrome: a pooled analysis.
        Circulation. 2016; 133: 622-630
        • Kyndt F.
        • Probst V.
        • Potet F.
        • et al.
        Novel SCN5A mutation leading either to isolated cardiac conduction defect or Brugada syndrome in a large French family.
        Circulation. 2001; 104: 3081-3086
        • Yang Y.
        • Hu D.
        • Sacher F.
        • et al.
        Meta-analysis of risk stratification of SCN5A with Brugada syndrome: is SCN5A always a marker of low risk?.
        Front Physiol. 2019; 10: 103
        • Milman A.
        • Gourraud J.B.
        • Andorin A.
        • et al.
        Gender differences in patients with Brugada syndrome and arrhythmic events: data from a survey on arrhythmic events in 678 patients.
        Heart Rhythm. 2018; 15: 1457-1465

      Linked Article

      • Letter to the Editor—Electrophysiological study in women with Brugada Syndrome
        Heart RhythmVol. 18Issue 6
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          The results of the study by Rodríguez-Mañero et al1 are in line with those reported by the two other most active groups in the field of Brugada syndrome (BrS).2,3 All three have confirmed the rare occurrence of malignant arrhythmic events (AEs), the lower prevalence of spontaneous type 1 BrS electrocardiogram (ECG), and the lower ventricular fibrillation (VF) inducibility rate in female patients with BrS. Similar to the Belgian2 and French3 reports, the Spanish study did not find that VF inducibility can be used for predicting arrhythmic risk in female patients.
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