Heart failure & cardiomyopathiesCohort study

Performance of the PRIMaCY sudden death risk prediction model for childhood hypertrophic cardiomyopathy: implications for implantable cardioverter-defibrillator decision-making

EuropacePublished

  • Retrospective cohort study
  • single-centre
  • Mean follow-up 4.9 (±3.8) years; median age 10 (IQR 4-14)

Authors' conclusion

Although PRIMaCY has a similar discriminatory ability to that reported for HCM Risk-Kids, estimated risk estimates did not correlate well with observed risk. A higher proportion of patients met implantable cardioverter-defibrillator thresholds using PRIMaCY model compared with HCM Risk-Kids.

Study context

Population
Children meeting diagnostic criteria for hypertrophic cardiomyopathy (HCM) in a large single-centre cohort
Participants
301 children
Exposure
PRIMaCY vs HCM Risk-Kids sudden cardiac death (SCD) risk models
Outcome
SCD or equivalent event; performance of estimated 5-year SCD risk

Main finding

As reported in the abstract

Model performance for 5-year SCD risk
GroupNNT to potentially treat one life-threatening arrhythmia
PRIMaCY clinical13.7
PRIMaCY genetic14.5
HCM Risk-Kids9.4
Harrell's C-statistic, clinical model
0.66 (95% CI 0.52-0.8)
Harrell's C-statistic, genetic model
0.66 (95% CI 0.54-0.80)
Calibration slope, clinical model
0.19 (95% CI 0.04-0.54)
Calibration slope, genetic model
0.26 (95% CI −0.03-0.62)

Observational data: associations, not effects.

Original publication

Performance of the PRIMaCY sudden death risk prediction model for childhood hypertrophic cardiomyopathy: implications for implantable cardioverter-defibrillator decision-making. Europace. Published 23 November 2023. doi:10.1093/europace/euad330

Summarised from the published abstract; every number is checked against it