Cardiovascular

Long-term predictors of outcome in Brugada syndrome patients after implantable cardioverter-defibrillator implant.

TL;DR

In 551 Brugada syndrome patients with an ICD followed for a median of 8.1 years, 11% had life-threatening arrhythmic events; ESC class IIa patients had a risk similar to secondary-prevention patients, and the BrS 5-year risk score (clinical plus genetic variables) at implant was associated with outcome, which may improve identification of patients who will benefit from ICD.

Key Findings

Eleven percent of ICD-implanted Brugada syndrome patients experienced life-threatening arrhythmic events (LAE) during long-term follow-up.

  • The cohort comprised 551 consecutive patients who received an ICD for primary or secondary prevention.
  • Median follow-up was 8.1 years.
  • Prospective data included arrhythmic history, ECG findings, therapies, SCN5A mutations, and genotyping of three BrS-associated single nucleotide variants.

Patients with an ESC class IIa indication for ICD had a risk profile similar to that of patients implanted for secondary prevention.

  • The abstract states that 'Patients with ESC class IIA indication had a similar risk profile as those implanted for secondary prevention.'
  • Guideline-based indications were assessed against actual arrhythmic events after ICD implant.

CPG class IIa and class I patients had a similar rate of ICD shock at follow-up.

  • The abstract reports that 'CPG class IIa and I had a similar rate of ICD shock at follow-up.'
  • This was evaluated in the context of the time-dependent expressivity of BrS.

The BrS 5-year risk score, which includes clinical and genetic variables, was higher in patients with LAEs than in those without, in the whole cohort and in primary-prevention patients.

  • Whole cohort: 26.93 ± 3.33 in patients with LAEs vs. 4.41 ± 0.61 in those without (P < 0.0001).
  • Primary-prevention patients: 11.67 ± 1.52 vs. 5.36 ± 0.99 (P < 0.0001).
  • The score was determined at the time of ICD implant.

Quantifying the BrS arrhythmic risk score at the time of ICD implant may improve identification of patients who will benefit from the ICD during follow-up.

  • The authors describe this as 'robust evidence' that the genetic substrate adds to prediction of arrhythmic events at follow-up.
  • The score incorporates clinical variables and genetic variables (SCN5A mutations and BrS-associated single nucleotide variants).

What This Means

This study followed 551 people with Brugada syndrome, an inherited heart rhythm condition, who had received an implantable cardioverter-defibrillator (ICD). Over a median of about 8 years, 11% had a life-threatening arrhythmic event. The aim was to see whether current guideline-based reasons for implanting an ICD actually predict who will have such events later, and whether genetic information adds to the prediction. Patients who met the European guideline class IIa criteria had a risk similar to those who received an ICD after a prior arrhythmic event (secondary prevention). Class IIa and class I patients also had similar rates of ICD shocks. A recently developed 5-year risk score that combines clinical and genetic information was clearly higher at the time of implant in people who later had life-threatening events than in those who did not, both across all patients and among those who received an ICD for primary prevention. This research suggests that calculating this risk score when an ICD is implanted may help identify which patients are most likely to benefit from the device over time. The findings come from an observational cohort of people who already had ICDs, so they do not address people without devices.

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Citation

Napolitano C, Pizzetti C, Kukavica D, Morini M, Ottaviano L, Giordano U, et al.. (2026). Long-term predictors of outcome in Brugada syndrome patients after implantable cardioverter-defibrillator implant.. Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology. https://doi.org/10.1093/europace/euag278