Heart failure & cardiomyopathies
External validation of the desmoplakin (DSP)-risk score for prediction of clinically significant ventricular arrhythmias in primary prevention patients with desmoplakin cardiomyopathy–associated genetic variants
Authors' conclusion
In a large independent cohort of DSP patients, this study showed external validity of the DSP-risk score. These findings support the use of the DSP-risk score to facilitate shared decision making regarding implantable cardioverter-defibrillator implantation in the primary prevention of VAs in patients harboring DSP P/LP variants.
Study context
- Population
- Primary prevention patients with pathogenic/likely pathogenic (P/LP) desmoplakin (DSP) variants with no sustained VA before or at first assessment, not used for development of the DSP-risk scoreDSP patients from 30 centers
- Participants
- 450 patientsEvents: 60
- Predictors
- DSP-risk score
- Predicted outcome
- sustained ventricular arrhythmias (VAs)
Methodological detailsinternational, multicenter
- International
- Multicenter
Model performance
As reported in the abstract
5-year risk
C-statistic
Overall
0.719(95% CI, 0.706-0.733)
With previous myocarditis-like episodes
0.719(95% CI, 0.702-0.737)
Without previous myocarditis-like episodes
0.749(95% CI, 0.740-0.759)
- Median follow-up 4.3 years (1.6-10.0)
Further findings
Baseline characteristics
- Mean age
- 42.2 ± 17.6
- Female
- 40.4%
- With previous myocarditis-like episode
- 18.4%
Original publication
External validation of the DSP-risk score for prediction of clinically significant ventricular arrhythmias in primary prevention patients with desmoplakin cardiomyopathy–associated genetic variants. Heart Rhythm. Published 27 April 2026. doi:10.
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