Arrhythmias & electrophysiology
Clinical profile and prognosis of brugada syndrome SCN5A variant carriers with negative sodium channel blocker challenge
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
Methodological detailsmulticentre, five university hospitals
- Multicentre
- Five university hospitals
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.
Summarised from the published abstract; every number is checked against it