Arrhythmias & electrophysiology
A novel tool for arrhythmic risk stratification in desmoplakin gene variant carriers
An erratum to this article was published (Eur Heart J, 14 Nov 2024 ↗)
Authors' conclusion
The DSP risk score is a novel model that leverages readily available clinical parameters to provide individualized VA risk assessment for DSP patients.
Study context
- Population
- Carriers of pathogenic or likely pathogenic desmoplakin (DSP) variants without prior sustained ventricular arrhythmia (VA) (DSP-ERADOS registry)71 with first sustained VA
- Participants
- 471 carriers
- Comparison
- Development vs External validation
- Developmentn = 385
- External validationn = 86
- Outcome
- First sustained VA event
- Follow-up
- Median 4.0 (IQR 1.6-7.3) years
Methodological detailsmultinational registry, longitudinal follow-up, step-wise Cox regression
- Multinational registry
- Longitudinal follow-up
- Step-wise Cox regression
Performance
As reported in the abstract
DSP risk score discrimination (c-statistic)
| Test or model | C-statistic | NPV in low-risk (<5% at 5 years; n = 26) |
|---|---|---|
| Development | .782 (95% CI: .77-.80) | — not stated in the abstract |
| External validation | .791 (95% CI: .75-.83) | 100% |
C-statistic
Development.782 (95% CI: .77-.80)
External validation.791 (95% CI: .75-.83)
NPV in low-risk (<5% at 5 years; n = 26)
Development— not stated in the abstract
External validation100%
— not stated in the abstract
Further findings
Independent predictors of VA (development cohort), included in DSP risk score
| HR (95% CI) | ||
|---|---|---|
| Female sex | 1.9 (1.1–3.4) | |
| History of non-sustained VT | 1.7 (1.1–2.8) | |
| Natural logarithm of 24-h premature ventricular contraction burden | 1.3 (1.1–1.4) | |
| LVEF < 50% | 1.5 (.95-2.5) | |
| Moderate to severe RV systolic dysfunction | 6.0 (2.9–12.5) | |
| 0.050.21520Hazard ratio (log scale) |
Incidence of first sustained VA · median 4.0 years follow-up
- Patients with first sustained VA events
- 71
- Event rate
- 2.6% events/year (95% CI 2.0, 3.5)
- n = 471
Original publication
A novel tool for arrhythmic risk stratification in desmoplakin gene variant carriers. Eur Heart J. Published 16 July 2024. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →