Arrhythmias & electrophysiology
Timing of cardioverter-defibrillator implantation in patients with cardiac laminopathies—External validation of the LMNA-risk ventricular tachyarrhythmia calculator
Authors' conclusion
Validation of the LMNA-risk VTA calculator showed high sensitivity for subsequent VTAs, but overestimated arrhythmic risk when using ≥7% predicted 5-year risk as threshold.
Study context
- Population
- LMNA genotype-positive patients without previous ventricular tachyarrhythmias (VTAs)
- Participants
- 118 patients
- Outcome
- Validation of the LMNA-risk VTA calculator (Harrell's C-statistic) for VTAs
- Follow-up
- Coming 5 years
Methodological detailscohort, 2 Scandinavian centres
- Cohort
- 2 Scandinavian centres
Performance
As reported in the abstract
Identification of patients with VTAs
| Test or model | Sensitivity | Specificity | Harrell's C-statistic |
|---|---|---|---|
| LMNA-risk VTA calculator | 83% | 26% | 0.85 |
LMNA-risk VTA calculator
Sensitivity83%
Specificity26%
Harrell's C-statistic0.85
Further findings
VTA incidence · 6.1 years (IQR 3.0-9.1 years) follow-up
- Patients experiencing VTA
- 23 (19%)
- Yearly incidence rate
- 3.0% (95% CI 2.0%-4.5%)
Predictors of VTA
- AV block and reduced LVEF were independent predictors; nonsustained VT, male sex and non-missense LMNA variants were not
Calculator sensitivity with risk reevaluated at last consultation before VTA
- Sensitivity
- 100%
Calibration by phenotype
- Calculator overestimated arrhythmic risk in mild and moderate phenotype, particularly in men
Original publication
Timing of cardioverter-defibrillator implantation in patients with cardiac laminopathies—External validation of the LMNA-risk ventricular tachyarrhythmia calculator. Heart Rhythm. Published 6 December 2022. doi:10.
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