Arrhythmias & electrophysiology
Long-term prognosis of patients with an SCN5A loss-of-function variant and progressive cardiac conduction disorder or Brugada syndrome
Authors' conclusion
Key predictors of arrhythmic risk in patients with an SCN5A loss-of-function variant, regardless of a Brugada syndrome diagnosis, are symptoms at baseline, male sex, and prolonged QRS interval.
Study context
- Population
- Probands and family members with (likely) pathogenic SCN5A loss-of-function variants (progressive cardiac conduction disorder or Brugada syndrome)242 probands
- Participants
- 615 participants
- Exposure
- Symptomatic vs Asymptomatic
- Symptomaticn = 111
- Outcome
- Arrhythmic events:
- Sudden death
- Aborted cardiac arrest
- Documented VF
- Sustained VT
- Follow-up
- Median 9.5 (Q1,Q3 5.0-14.3) years
Main finding
As reported in the abstract
Arrhythmic events · median follow-up 9.5 years
Symptomatic
2.0%/y
Asymptomatic
0.3%/y
- Arrhythmic events overall
- 41 patients (6.7%)
- Overall event rate
- 0.7%/y
Observational data: associations, not effects.
Further findings
- Male sex and QRS prolongation also identified as predictors
- Asymptomatic women with QRS interval < 100 ms did not experience arrhythmic events at follow-up
Original publication
Long-term prognosis of patients with an SCN5A loss-of-function variant and progressive cardiac conduction disorder or Brugada syndrome. Heart Rhythm. Published 2 November 2024. doi:10.
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