Heart failure & cardiomyopathies
Independent External Evaluation of Pediatric Hypertrophic Cardiomyopathy Risk Scores in Predicting Severe Ventricular Arrhythmias
Authors' conclusion
In this independent evaluation, our findings suggest imperfect discrimination between low and high-risk patients using the HCM Risk-Kids and PRIMaCY risk scores, with predicted risks tending to be overestimated compared with the actual observed events. The performance or risk scores was substantially improved by periodic reassessment during follow-up.
Study context
- Population
- Patients with hypertrophic cardiomyopathy (HCM) aged <18 years, primary prevention children, 2003–2023
- Participants
- 100 primary prevention childrenEvents: 13 (13.0%)
- Predictors
- HCM Risk-Kids and PRIMaCY risk scores
- Predicted outcome
- Primary composite outcome:
- Sustained ventricular arrhythmia
- Appropriate implantable cardioverter defibrillator (ICD) therapy
- Aborted cardiac arrest
- Sudden cardiac death
Model performance
As reported in the abstract
5 first years
Harrel C index
HCM Risk-Kids (≥6%)
0.52(95% CI, 0.27-0.77)
PRIMaCY (>8.3%)
0.70(95% CI, 0.59-0.80)
| Test or model | Number of implantable cardioverter defibrillator (ICDs) implanted per patient potentially treated |
|---|---|
| HCM Risk-Kids (≥6%) | 25 |
| PRIMaCY (>8.3%) | 14 |
Number of implantable cardioverter defibrillator (ICDs) implanted per patient potentially treated
HCM Risk-Kids (≥6%)25
PRIMaCY (>8.3%)14
Further findings
| Outcome | HCM Risk-Kids | PRIMaCY |
|---|---|---|
| Primary outcomes during all follow-up, risk scores repeated | ||
| Number of ICDs implanted per patient potentially treated | 5.6 | 5.3 |
- Primary outcomes during all follow-up, risk scores repeated — Events correctly identified using both risk scores
- 12 of 13 (92.3%)
Appropriate ICD therapy among patients implanted with an ICD
- Patients implanted with an ICD
- 44 (44.0%)
- All primary prevention patients with ≥1 appropriate ICD therapy had HCM Risk-Kids ≥6% and PRIMaCY >8.3% at implantation
Follow-up
- Mean follow-up
- 8.6±5.5 years
Original publication
Independent External Evaluation of Pediatric Hypertrophic Cardiomyopathy Risk Scores in Predicting Severe Ventricular Arrhythmias. Circ Arrhythm Electrophysiol. Published 20 February 2025. doi:10.
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