Background <p>The Shanghai score system was developed to enhance the&#xa0;risk stratification in Brugada Syndrome (BrS); however, its prognostic value in drug-induced type 1 BrS remains unclear.</p> Methods <p>This study involved 698 patients with drug-induced type 1 BrS, confirmed via pharmacologic challenge (flecainide or ajmaline), from 21 centers in Italy and Switzerland. Patients were classified according to the Shanghai score system: probable/definite BrS (score ≥ 3.5) and possible BrS (score &lt; 3.5). The primary outcome was appropriate ICD therapy or sudden cardiac death (SCD)/sustained ventricular arrhythmias; the secondary outcome includes the identification of clinical predictors of primary outcome events. Kaplan–Meier and Cox regression analyses were used.</p> Results <p>Our study population included 239 patients (34.2%) with probable/definite BrS and 459 (65.8%) patients with possible BrS. During a median follow-up of 57.4&#xa0;months, 20 patients (2.9%) experienced the primary outcome. Kaplan–Meier analysis revealed a significantly lower event rate in possible BrS (0.11% over 10&#xa0;years) compared to probable/definite BrS (0.42%). <i>SCN5A</i> pathogenic variants were a significant predictor of primary endpoint in the possible BrS group (OR: 12.5).</p> Conclusions <p>Shanghai score system for BrS diagnosis may be useful as a tool for risk stratification of life-threatening arrhythmias among patients with drug-induced type I BrS ECG. Identifying the <i>SCN5A</i> mutations is of pivotal importance for refining the risk stratification.</p> Graphical Abstract <p></p>

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Predictive value of Shanghai score system in patients with drug-induced type 1 Brugada electrocardiographic pattern

  • Vincenzo Russo,
  • Alfredo Caturano,
  • Federico Migliore,
  • Federico Guerra,
  • Pietro Francia,
  • Martina Nesti,
  • Giulio Conte,
  • Alessandro Paoletti Perini,
  • Giuseppe Mascia,
  • Stefano Albani,
  • Procolo Marchese,
  • Vincenzo Ezio Santobuono,
  • Gregory Dendramis,
  • Andrea Rossi,
  • Andrea Ottonelli Ghidini,
  • Pasquale Notarstefano,
  • Luigi Sciarra,
  • Zefferino Palamà,
  • Enrico Baldi,
  • Roberto Floris,
  • Gerardo Nigro,
  • Emilio Attena,
  • Emanuele Romeo,
  • Antonio D’Onofrio,
  • Carmen Adducci,
  • Livia Pardo Franchetti,
  • Nicola Berlier,
  • Berardo Sarubbi,
  • Ferdinando Carlo Sasso,
  • Paolo Golino,
  • Alessandro Vicentini,
  • Luca Barca,
  • Italo Porto,
  • Nicolò Martini,
  • Chiara Carrozzi,
  • Gianfranco Tola

摘要

Background

The Shanghai score system was developed to enhance the risk stratification in Brugada Syndrome (BrS); however, its prognostic value in drug-induced type 1 BrS remains unclear.

Methods

This study involved 698 patients with drug-induced type 1 BrS, confirmed via pharmacologic challenge (flecainide or ajmaline), from 21 centers in Italy and Switzerland. Patients were classified according to the Shanghai score system: probable/definite BrS (score ≥ 3.5) and possible BrS (score < 3.5). The primary outcome was appropriate ICD therapy or sudden cardiac death (SCD)/sustained ventricular arrhythmias; the secondary outcome includes the identification of clinical predictors of primary outcome events. Kaplan–Meier and Cox regression analyses were used.

Results

Our study population included 239 patients (34.2%) with probable/definite BrS and 459 (65.8%) patients with possible BrS. During a median follow-up of 57.4 months, 20 patients (2.9%) experienced the primary outcome. Kaplan–Meier analysis revealed a significantly lower event rate in possible BrS (0.11% over 10 years) compared to probable/definite BrS (0.42%). SCN5A pathogenic variants were a significant predictor of primary endpoint in the possible BrS group (OR: 12.5).

Conclusions

Shanghai score system for BrS diagnosis may be useful as a tool for risk stratification of life-threatening arrhythmias among patients with drug-induced type I BrS ECG. Identifying the SCN5A mutations is of pivotal importance for refining the risk stratification.

Graphical Abstract