Arrhythmias & electrophysiology
Type 3 long QT syndrome: Is the effectiveness of treatment with beta-blockers population-specific?
Authors' conclusion
In agreement with the literature, proband status and lengthened QTc were associated with a higher risk of CEs. Our data do not show a protective effect of beta-blocker treatment.
Study context
- Population
- French cohort of LQT3 patients with a likely pathogenic/pathogenic SCN5A variant54 families
- Participants
- 147 participants
- Exposure
- Beta-blocker vs no beta-blocker
- Outcome
- CEs (VT/VF, torsades de pointes, aborted cardiac arrest, sudden death, appropriate shocks, syncope) and SCEs (CEs excluding syncope)
Methodological detailsMedian follow-up 11 years; 6 patients symptomatic before age 1 year analysed separately
- Median follow-up 11 years; 6 patients symptomatic before age 1 year analysed separately
Main finding
As reported in the abstract
Age at first CE/SCE, by beta-blocker treatment
- Cumulative probability of age at first CE/SCE not lower with beta-blocker
Observational data: associations, not effects.
Further findings
Predictors of cardiac events (CEs)
| HR (CI) | P value | ||
|---|---|---|---|
| QTc prolongationIndependently associated | 1.12 (1.0–1.2) | P = 0.005 | |
| Proband statusIndependently associated | 4.07 (1.9–8.9) | P < 0.001 | |
| 0.10.31310Hazard ratio (log scale) |
Predictors of severe cardiac events (SCEs)
| HR (CI) | P value | ||
|---|---|---|---|
| Proband status | 8.13 (1.7–38.8) | P = 0.009 | |
| QTc prolongation | 1.11 (1.0–1.3) | P = 0.108 | |
| 0.050.21520Hazard ratio (log scale) |
Probability of CE and SCE from birth to age 40
- CE
- 20.5%
- SCE
- 9.9%
Original publication
Type 3 long QT syndrome: Is the effectiveness of treatment with beta-blockers population-specific? Heart Rhythm. Published 11 November 2023. doi:10.
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