Arrhythmias & electrophysiologyCohort study
Clinical profile and prognosis of brugada syndrome SCN5A variant carriers with negative sodium channel blocker challenge
- Retrospective cohort study
- multicentre
- five university hospitals
Authors' conclusion
This multicentre study provides the largest analysis of SCN5A carriers with negative SCBC, showing excellent arrhythmic prognosis despite frequent progressive conduction slowing.
Study context
- Population
- Carriers of a pathogenic or likely pathogenic SCN5A variant (Brugada syndrome (BrS)), five university hospitals, 2000–2024; spontaneous type 1 ECG, gain-of-function, double or ACMG class 1–3 variants excluded43 families
- Participants
- 162 carriers
- Exposure
- SCN5A variant carriers with negative vs positive sodium channel blocker challenge (SCBC)
- Negative SCBCn = 69
Main finding
As reported in the abstract
Conduction defects and ICD implantation · median follow-up 75 [40-168] months
Conduction defects
P = 0.002
ICD implantations
P < 0.001
Observational data: associations, not effects.
Further findings
| Outcome | P value |
|---|---|
| Conduction slowing, non-missense vs missense variants · 71% vs. 42% | P = 0.04 |
Baseline conduction abnormalities (negative SCBC)
- Intraventricular conduction defects
- 25 (36%)
- First-degree AV block
- 19 (28%)
Progressive conduction slowing (whole cohort) · median follow-up 75 [40-168] months
- Developed progressive conduction slowing
- 52%
Original publication
Clinical profile and prognosis of brugada syndrome SCN5A variant carriers with negative sodium channel blocker challenge. Europace. Published 11 March 2026. doi:10.1093/europace/euag014
Summarised from the published abstract; every number is checked against it